Breast

Inflammatory

Mammary Duct Ectasia / Periductal Mastitis

Acute Mastitis

Lymphocytic & Diabetic Mastitis

Cystic neutrophilic granulomatous mastitis

Silicone mastitis / Granuloma

Developmental Disorders

Juvenile Papillomatosis

Gynecomastia

Mammary hamartoma

Benign Epithelial

Columnar Cell Change (CCC)

Columnar Cell Hyperplasia (CCH)

Flat Epithelial Atypia (FEA)

Usual Ductal Hyperplasia (UDH)

Microglandular Adenosis (MGA)

Sclerosing Adenosis

Apocrine Adenosis

Collagenous Spherulosis

Radial Scar / Complex Sclerosing Lesion (CSL)

Lactating Adenoma

Tubular Adenoma

Epithelial-myoepithelial tumors

Pleomorphic adenoma

Adenomyoepithelioma

Papillary Lesions and Neoplasia

Intraductal papilloma

Papillary DCIS

Solid Papillary Carcinoma (SPC)

Encapsulated Papillary Carcinoma (EPC)

Lobular Neoplasia

Atypical Lobular Hyperplasia (ALH)

Lobular Carcinoma In Situ (LCIS)

Ductal Neoplasia

Atypical Ductal Hyperplasia (ADH)

Ductal Carcinoma In Situ (DCIS)

Invasive breast carcinoma

Microinvasive carcinoma

Invasive lobular carcinoma (ILC)

Tubular Carcinoma

Tubulolobular Carcinoma

Invasive Cribriform Carcinoma

Mucinous Carcinoma

Invasive Carcinoma with Medullary Features

Papillary Carcinoma (Invasive)

Invasive Micropapillary Carcinoma

Invasive Carcinoma with Apocrine Differentiation

Apocrine Carcinoma

Metaplastic Carcinoma

Rare and salivary gland-type tumors

Acinic Cell Carcinoma

Adenoid Cystic Carcinoma

Secretory Carcinoma

Mucoepidermoid Carcinoma

Tall Cell Carcinoma with Reversed Polarity (Pattern)

Breast ─ Fibroepithelial & Mesenchymal

Fibroadenoma

Phyllodes Tumor

Mesenchymal Tumors

Hemangioma

Angiomatosis

Angiosarcoma

Nodular Fasciitis

Myofibroblastoma

Fibromatosis (Desmoid Tumor)

Granular Cell Tumor

Pseudoangiomatous Stromal Hyperplasia (PASH)

Inflammatory

Mammary Duct Ectasia / Periductal Mastitis

Dilatation of major subareolar ducts associated with periductal inflammation and fibrosis.

Clinical  Often smokers; thick nipple discharge, nipple retraction, subareolar mass.

Radiology ─

 Mammography: May show dilated retroareolar ducts, +/- microcalcifications (often linear, coarse within ducts)

 Ultrasound: May show dilated ducts with echogenic debris or fluid, duct wall thickening

Macro  Dilated ducts containing thick, cheesy, or pasty material (inspissated secretions); surrounding tissue may be firm/fibrotic

Micro ─

 Dilated large ducts, often filled with eosinophilic, proteinaceous secretions and foamy macrophages (lipid-laden)

 Periductal chronic inflammation (lymphocytes, plasma cells - often prominent)

 Periductal fibrosis, variable degrees

 +/- Squamous metaplasia of duct lining

 +/- Cholesterol clefts, calcifications within duct lumen or wall

 +/- Granulomatous inflammation (foreign body reaction to secretions)

 In later stages, ducts may be obliterated by fibrosis

DDx ─

 DCIS (Comedo type) (Necrosis central, atypia present, lacks prominent plasma cells/foamy macrophages in duct)

 Plasma Cell Mastitis (Term often used interchangeably or for cases dominated by plasma cells)

 Invasive Carcinoma (Infiltrative growth pattern, cytologic malignancy)

 Abscess (Acute inflammation, neutrophils predominate)

 Specific granulomatous mastitis (e.g., TB, fungal) (Specific organisms, well-formed granulomas)

Prognosis  Benign inflammatory condition; may resolve, persist, or recur; main significance is mimicry of malignancy

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Acute Mastitis

Acute inflammation of the breast, typically bacterial, often associated with lactation.

Clinical  Lactating women (puerperal mastitis); pain, swelling, erythema, warmth, fever; may form abscess

Radiology ─

 Ultrasound: ill-defined hypoechoic area with increased vascularity, skin thickening, +/- fluid collection/abscess

Macro  Affected area is indurated, erythematous; abscess appears as fluctuant mass with purulent material

Micro ─

 Dense neutrophilic infiltrate within lobules and stroma

 Tissue necrosis, edema, hemorrhage

 Abscess formation (collection of pus)

 +/- granulation tissue, reactive epithelial changes in later stages

DDx ─

 Inflammatory Breast Carcinoma (Dermal lymphatic invasion by tumor cells, clinical presentation)

 Other forms of mastitis (Granulomatous, Lymphocytic - different inflammatory infiltrate)

 Mammary Duct Ectasia/Periductal Mastitis (Plasma cells prominent, duct dilation with foamy macrophages)

Prognosis  Benign inflammatory condition; usually resolves with antibiotics; abscess may require drainage

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Lymphocytic & Diabetic Mastitis

Uncommon condition, dense lymphocytic infiltrates and keloidal fibrosis, strongly associated with type 1 diabetes mellitus. Also known as Sclerosing Lymphocytic Lobulitis.

Clinical  Typically premenopausal women with long-standing type 1 diabetes (can also occur in type 2 DM or autoimmune thyroid disease); presents as hard, painless, often bilateral breast mass(es); may mimic malignancy clinically and radiologically

Radiology ─

 Mammography: Ill-defined density or asymmetry

 Ultrasound: Ill-defined hypoechoic mass with posterior acoustic shadowing, mimicking carcinoma

Macro  Ill-defined, very firm, rubbery, gray-white tissue

Micro ─

 Dense keloidal fibrosis (thick, hyalinized collagen bundles)

 Lobulocentric lymphocytic infiltrate (predominantly B-cells, some T-cells and plasma cells) surrounding atrophic ducts and lobules

 Lymphocytic vasculitis (lymphocytes infiltrating vessel walls) may be present

 Epithelioid stromal fibroblasts (plump fibroblasts with eosinophilic cytoplasm) may be seen

 +/- Diabetic microangiopathy in adjacent tissue

DDx ─

 Invasive Carcinoma (esp. Lobular) (Malignant cells present, lacks prominent keloidal fibrosis/lymphocytic vasculitis)

 Fibromatosis (Lacks dense lobulocentric lymphoid infiltrate/vasculitis, β-catenin+)

 Sclerosing Lobular Hyperplasia (Less inflammation, lacks keloidal fibrosis/vasculitis)

 Nodular Sclerosis Hodgkin Lymphoma (Reed-Sternberg cells present, specific IHC)

 Other forms of mastitis (Lack keloidal fibrosis and prominent B-cell rich infiltrate)

Prognosis  Benign inflammatory condition; tends to recur; increased risk of subsequent lymphoma not established

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Cystic neutrophilic granulomatous mastitis

Rare form of granulomatous mastitis with cystic spaces containing neutrophils and associated with Corynebacterium species.

Clinical  Women of reproductive age, often history of smoking, parity, or nipple piercing; presents as tender, erythematous breast mass, may form abscess or fistula

Radiology ─

 Ultrasound/Mammography: May show ill-defined mass, abscess formation, complex cystic structures

Macro  Ill-defined inflammatory mass, may show abscess/cyst formation

Micro ─

 Lobulocentric inflammation

 Characteristic feature: Clear lipid vacuoles/spaces within granulomas or suppurative areas

 Vacuoles are lined by neutrophils and/or histiocytes

 Gram-positive bacilli (Corynebacterium) often demonstrable within vacuoles (requires Gram stain)

 Mixed inflammatory infiltrate (neutrophils, lymphocytes, plasma cells, histiocytes, giant cells)

 +/- Duct ectasia, abscess formation, fibrosis

DDx ─

 Idiopathic Granulomatous Mastitis (Lacks cystic spaces with neutrophils/Corynebacterium)

 Other infectious granulomatous mastitis (TB, fungal - specific organisms identified)

 Mammary Duct Ectasia (Plasma cells prominent, foamy macrophages in ducts, lacks vacuoles with neutrophils)

 Fat Necrosis (History of trauma/surgery, different morphology)

Prognosis  Benign inflammatory condition; often requires prolonged antibiotics; may recur

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Silicone mastitis / Granuloma

Granulomatous inflammatory reaction to extravasated silicone, usually from implant rupture or direct injection.

Clinical  History of breast implants (ruptured or leaking) or direct silicone injection; presents as firm nodules, masses, or diffuse induration; may have pain, skin changes, lymphadenopathy

Radiology ─

 Ultrasound: "Snowstorm" appearance (diffuse high echogenicity with obscured posterior structures)

 MRI: Characteristic silicone signal; can identify intracapsular vs extracapsular rupture

Macro  Firm, nodular, or diffusely indurated tissue; may see glistening, refractile material (silicone)

Micro ─

 Numerous cystic spaces/vacuoles of varying sizes (representing dissolved silicone) within fibrous stroma or adipose tissue

 Surrounded by prominent foreign body giant cell reaction (multinucleated giant cells engulfing silicone)

 Chronic inflammatory infiltrate (lymphocytes, plasma cells, histiocytes - some foamy)

 Dense fibrosis common

 Silicone material itself is refractile but non-birefringent (may be seen if processing doesn't dissolve it)

DDx ─

 Fat Necrosis (Different type of vacuoles - adipocytes, lacks refractile material, often history of trauma/surgery)

 Other forms of granulomatous mastitis (Lack silicone vacuoles/history)

 Cystic Neutrophilic Granulomatous Mastitis (Neutrophils lining vacuoles, Corynebacterium)

Prognosis  Benign inflammatory reaction; can cause significant fibrosis and cosmetic deformity; silicone lymphadenopathy can occur

Media  WSI WSI

Developmental Disorders

Juvenile Papillomatosis

Benign proliferative lesion occurring in young women, with duct papillomatosis, cysts, epithelial hyperplasia, and sclerosing adenosis. Also known as "Swiss cheese disease" due to cystic appearance.

Clinical  Adolescents and young women (typically < 30 years); presents as palpable, often well-circumscribed mass

Radiology ─

 Mammography/Ultrasound: Often shows ill-defined mass with multiple small cysts

Macro  Poorly circumscribed, firm mass with prominent small cysts ("Swiss cheese")

Micro ─

 Florid duct papillomatosis (intraductal papillary proliferation)

 Multiple cysts, often with apocrine metaplasia

 Usual ductal hyperplasia (UDH), often florid

 Sclerosing adenosis

 Stroma may be fibrotic

 Atypia (ADH/ALH) or DCIS can occasionally be present concurrently or arise later

DDx ─

 Fibrocystic changes (Less florid papillomatosis/hyperplasia, usually older patients)

 Multiple papillomas (Discrete papillary tumors, less background hyperplasia/sclerosis)

 DCIS (Papillary/Cribriform) (Significant cytologic atypia, monomorphic population, lacks prominent cysts/sclerosis typical of JP)

 Fibroadenoma (Juvenile) (More prominent stromal component, different architecture)

Prognosis  Benign, but considered a marker of increased risk for subsequent breast cancer development (both ipsilateral and contralateral), especially if associated with atypia or family history

Media  WSI

Gynecomastia

Benign enlargement of the male breast due to proliferation of ducts and stroma.

Clinical  Can occur at any age (neonatal, puberty, older age); associated with hormonal imbalance (relative estrogen excess), medications (e.g., spironolactone, cimetidine, hormones), liver disease, Klinefelter syndrome, tumors (testicular, adrenal); presents as palpable, often tender, subareolar mass/disc, unilateral or bilateral

Radiology ─

 Mammography: Typically shows retroareolar density, may be flame-shaped or nodular

Macro  Rubbery, firm, gray-white tissue in subareolar region

Micro ─

 Variable proliferation of both ducts and stroma around the nipple

 Florid phase (early): Epithelial hyperplasia within ducts (often micropapillary tufts, UDH-like), periductal stromal edema, increased vascularity, mild inflammation

 Fibrous phase (late): Dense stromal fibrosis, fewer ducts which may be atrophic or dilated, less epithelial hyperplasia

 +/- Squamous metaplasia, apocrine metaplasia, pseudoangiomatous stromal hyperplasia (PASH)

 Lobule formation is absent (key feature distinguishing from female breast)

DDx ─

 Male Breast Carcinoma (Invasive or DCIS) (Cytologic atypia, infiltrative growth, necrosis, lacks lobular architecture)

 Pseudogynecomastia (Adipose tissue deposition without ductal/stromal proliferation)

 Other benign male breast lesions (rare - papilloma, fibroadenoma-like changes)

Prognosis  Benign; no increased risk for male breast cancer

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Mammary hamartoma

Benign, circumscribed lesion composed of mature but disorganized breast tissue elements (lobules, ducts, adipose tissue, fibrous stroma). Also known as fibroadenolipoma.

Clinical  Adults (wide age range, mc > 40 years), F >> M; presents as painless, soft to firm, mobile mass; often well-circumscribed ("breast within a breast" on imaging)

Radiology ─

 Mammography: Characteristically well-circumscribed, encapsulated mass containing mixed densities of fat and fibroglandular tissue ("breast within a breast")

 Ultrasound: Heterogeneous encapsulated mass with mixed echogenicity

Macro  Well-encapsulated, round to oval mass; cut surface shows lobulated gray-white fibrous/glandular tissue admixed with yellow adipose tissue

Micro ─

 Encapsulated (or sharply circumscribed) lesion

 Disorganized mixture of benign breast elements:

 Mature lobules and ducts (may show usual hyperplasia, cysts, apocrine metaplasia)

 Fibrous stroma (often hypocellular)

 Mature adipose tissue

 Relative proportions of components vary widely

 Pseudoangiomatous stromal hyperplasia (PASH) common within stroma

 Myoid hamartoma variant contains smooth muscle

DDx ─

 Fibroadenoma (Biphasic, lacks mature lobules/adipose tissue within the lesion proper, different stromal quality)

 Lipoma (Pure adipose tissue)

 Normal breast tissue (Lacks encapsulation/sharp circumscription and disorganized arrangement)

 Phyllodes Tumor (Leaf-like architecture, increased stromal cellularity/atypia)

Prognosis  Benign; excision is curative

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Benign Epithelial

Columnar Cell Change (CCC)

Alteration of terminal duct lobular units (TDLUs) with replacement of native cuboidal epithelium with one or two layers of taller columnar cells, often with apical snouts and intraluminal secretions/calcifications. Considered benign.

Clinical  Common incidental finding on biopsy, often associated with microcalcifications; wide age range

Radiology ─

 Mammography: amorphous, punctate, or pleomorphic microcalcifications, typically grouped

Macro  Not grossly distinct

Micro ─

 Enlarged TDLUs with dilated acini/ductules

 Lined by 1-2 layers of columnar epithelial cells (taller than normal cuboidal cells)

 Apical cytoplasmic snouts/blebs common

 Intraluminal secretions (eosinophilic, proteinaceous)

 Microcalcifications (calcium oxalate or phosphate) frequent within lumens

 Minimal cytologic atypia (nuclei usually small, round/oval, normochromatic)

 Myoepithelial layer intact

IHC ─ (+) ER (often strong/diffuse), PR (variable); (+/-) CK5/6 (may show mosaic pattern if >1 layer); (-) HER2

DDx ─

 Apocrine metaplasia (Granular eosinophilic cytoplasm, prominent nucleoli, AR+, GCDFP-15+)

 Columnar Cell Hyperplasia (CCH) (>2 cell layers)

 Flat Epithelial Atypia (FEA) (Cytologic atypia present)

 Atypical Ductal Hyperplasia (ADH) / DCIS (More complex architecture, cytologic atypia)

Prognosis  Benign; considered non-proliferative or mildly proliferative; minimal to no increased risk for breast cancer on its own

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Columnar Cell Hyperplasia (CCH)

Alteration of TDLUs with proliferation of columnar epithelial cells (>2 cell layers), often with tufting or micropapillations, but lacking significant cytologic atypia.

Clinical  Common incidental finding on biopsy, often associated with microcalcifications; wide age range

Radiology ─

 Mammography: amorphous, punctate, or pleomorphic microcalcifications, typically grouped

Macro  Not grossly distinct

Micro ─

 Similar to CCC (enlarged TDLUs, dilated acini, apical snouts, secretions, calcifications)

 Epithelial lining is >2 cell layers thick

 May show cellular crowding, stratification, tufting, or simple micropapillations projecting into lumen

 Lacks significant cytologic atypia (nuclei generally bland, similar to CCC)

 Myoepithelial layer intact

IHC ─ (+) ER (often strong/diffuse), PR (variable); (+/-) CK5/6 (often shows mosaic pattern); (-) HER2

Molecular  Clonal alterations reported but significance unclear

DDx ─

 Columnar Cell Change (CCC) (2 cell layers)

 Flat Epithelial Atypia (FEA) (Cytologic atypia present)

 Usual Ductal Hyperplasia (UDH) (Different cytology - overlapping/streaming nuclei, indistinct borders, slit-like spaces; often mosaic CK5/6)

 Atypical Ductal Hyperplasia (ADH) / DCIS (More complex architecture, cytologic atypia)

Prognosis  Benign proliferative lesion; may confer a small increased risk for breast cancer, similar to mild UDH

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Flat Epithelial Atypia (FEA)

An alteration of terminal duct lobular units (TDLUs) where native epithelium is replaced by one to several layers of monotonous, mildly atypical columnar to cuboidal cells, lacking complex architectural features like bridging or micropapillae. Considered a low-risk atypical lesion.

Clinical  Often an incidental finding associated with microcalcifications on mammography; wide age range, but more common in perimenopausal/postmenopausal women

Radiology ─

 Mammography: associated with amorphous or punctate microcalcifications, often grouped

Macro  Not grossly distinct

Micro ─

 Involves TDLUs, replacing native epithelium

 One or more layers (often 1-3) of monotonous epithelial cells

 Cells cuboidal to columnar, may have apical snouts

 Cytologic atypia: Mildly enlarged, round to oval nuclei; hyperchromatic or vesicular chromatin; small nucleoli may be visible; minimal pleomorphism

 Loss of polarity compared to normal epithelium

 Lumens often contain secretions and calcifications

 Lacks complex architecture (no significant bridging, micropapillae, or solid growth)

 Myoepithelial layer intact

IHC ─ (+) ER (strong, diffuse); (+) CK8/18; (-) CK5/6 (negative or only scattered positive cells); (-) HER2

Molecular  alterations common (16q loss, 17p gain), overlap with low-grade DCIS and tubular carcinoma

DDx ─

 Columnar Cell Change/Hyperplasia (CCC/CCH) (Lacks cytologic atypia)

 Atypical Ductal Hyperplasia (ADH) (More complex architecture - micropapillae, rigid bridges, cribriform spaces)

 DCIS, Low Grade (esp. cribriform/micropapillary) (More complex architecture, greater cytologic atypia)

 Apocrine Metaplasia (Different cytology - eosinophilic granular cytoplasm, prominent nucleoli, AR+)

Prognosis  Considered a low-risk lesion, but associated with a slightly increased risk of subsequent breast cancer (similar to ADH); often found adjacent to ADH, DCIS, or invasive carcinoma (esp. tubular)

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Usual Ductal Hyperplasia (UDH)

Benign intraductal proliferation with an increased number of epithelial cells filling and distending ducts/lobules; shows architectural and cytologic heterogeneity.

Clinical  Very common finding, often incidental; may be associated with calcifications or palpable masses related to fibrocystic changes; wide age range, common in reproductive/perimenopausal years

Radiology ─

 Mammography: benign-appearing calcifications (punctate, amorphous)

Micro ─

 Increased number of cells (>2 layers) filling part or all of a duct or lobular unit lumen

 Cells often arranged haphazardly, showing streaming and overlapping nuclei

 Irregular, slit-like, peripherally located secondary lumens common ("fenestrated" pattern)

 Mixed cell population: Epithelial cells, myoepithelial cells, metaplastic cells intermingled

 Nuclei vary in size/shape, often oval or spindled, chromatin fine to slightly coarse, nucleoli inconspicuous

 Indistinct cell borders

 Lacks significant cytologic atypia or architectural monotony seen in ADH/DCIS

 Myoepithelial layer surrounding involved duct/lobule is intact

IHC ─

 (+) CK5/6 or other HMWK (shows characteristic "mosaic" or mixed staining pattern, highlighting heterogeneity)

 (+) ER, PR (often heterogeneous/patchy staining intensity and distribution)

 (-) HER2

 (+) Myoepithelial markers (p63, SMA etc.) show intact peripheral layer

Molecular  polyclonal

DDx ─

 Atypical Ductal Hyperplasia (ADH) (Monomorphic population, distinct architectural patterns like cribriform/micropapillary, lacks CK5/6 mosaicism, strong/diffuse ER)

 DCIS, Low Grade (More pronounced atypia/monotony, distinct architecture, lacks CK5/6 mosaicism)

 Intraductal Papilloma (True fibrovascular cores lined by epithelium and myoepithelium)

 Normal Duct/Lobule (Only 1-2 cell layers)

Prognosis  Benign proliferative lesion; confers a small increased risk (relative risk ~1.5-2x) for subsequent development of breast cancer

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Microglandular Adenosis (MGA)

Rare type of adenosis with a haphazard proliferation of small, uniform, round glands lacking a myoepithelial layer, often containing eosinophilic secretions.

Clinical  Wide age range; may present as palpable mass, mammographic density, or calcifications

Radiology ─ Non-specific; may show density, distortion, or calcifications

Macro  Ill-defined, firm, gray-white area

Micro ─

 Haphazard proliferation of small, round, open glands infiltrating stroma and adipose tissue

 Glands lined by a single layer of cuboidal cells with bland nuclei, often clear or eosinophilic cytoplasm

 Characteristic feature: Absence of myoepithelial layer (confirmed by IHC)

 Lumens often contain dense, eosinophilic, colloid-like secretions (PAS+)

 Intact basement membrane surrounds glands (collagen IV+, laminin+)

 Minimal stromal response

IHC ─ (+) S100 (strong, diffuse in epithelial cells); (+) CK8/18; (-) ER, PR, HER2 (typically triple negative);(-) Myoepithelial markers (p63, SMA, etc.)

DDx ─

 Tubular Carcinoma (Angulated/teardrop glands, infiltrative growth with desmoplasia, lacks S100, ER+, lacks eosinophilic secretions)

 Sclerosing Adenosis (Retains myoepithelial layer, glands often compressed/distorted)

 Apocrine Adenosis (Apocrine cytology, retains myoepithelial layer)

 Secretory Carcinoma (Different cytology, intracellular/extracellular secretions, ETV6::NTRK3 fusion)

Prognosis  Benign itself, but considered non-obligate precursor for carcinoma (often triple negative invasive carcinoma can arise in MGA)

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Sclerosing Adenosis

Common type of adenosis with proliferation of acini and stromal fibrosis, leading to compression and distortion of the glandular elements, but maintaining a lobular architecture at low power.

Clinical  Common component of fibrocystic changes; may present as palpable mass or mammographic density/calcifications; wide age range

Radiology ─

 Mammography: May show microcalcifications (often punctate, amorphous, sometimes pleomorphic), density, or architectural distortion; can mimic carcinoma

Macro  Firm, rubbery, ill-defined area

Micro ─

 Lobulocentric proliferation (overall lobular shape maintained at low power)

 Increased number of small acini/ductules, often compressed and distorted by dense fibrous stroma

 Glands lined by epithelial and myoepithelial cells

 Myoepithelial layer is intact, often prominent

 Streaming and swirling of epithelial cells common

 Microcalcifications frequent within lumens

 May extend into surrounding stroma/fat ("adenosis tumor" - still benign)

IHC ─ Myoepithelial markers (p63, SMA, calponin, CK5/6) highlight intact myoepithelial layer, confirming benignity; ER/PR variable

DDx ─

 Tubular Carcinoma (Infiltrative growth, angulated glands, lacks myoepithelial layer, desmoplastic stroma, ER+)

 Invasive Lobular Carcinoma (Single file infiltration, lacks myoepithelial layer, E-cadherin-)

 Microglandular Adenosis (Lacks myoepithelial layer, S100+, eosinophilic secretions)

 Radial Scar (Different architecture - central fibroelastotic core)

Prognosis  Benign proliferative lesion; confers a minimal increased risk (relative risk ~1.5-2x) for subsequent breast cancer

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Apocrine Adenosis

Adenosis (usually sclerosing type) in which the epithelial cells show prominent apocrine metaplasia.

Clinical  Component of fibrocystic changes; presentation similar to sclerosing adenosis

Radiology ─ Similar to sclerosing adenosis, often associated with calcifications

Macro  Not grossly distinct

Micro ─

 Features of adenosis (increased acini, often compressed/distorted by fibrosis, lobulocentric)

 Epithelial cells lining the acini show apocrine metaplasia (abundant granular eosinophilic cytoplasm, round nuclei, prominent nucleoli, apical snouts)

 Myoepithelial layer intact

 May show mild nuclear atypia ("atypical apocrine adenosis" - still considered benign if architecture is adenosis)

IHC ─ Epithelial cells (+) GCDFP-15, AR; (-) ER, PR; Myoepithelial markers (+)

DDx ─

 Apocrine DCIS involving adenosis (Significant cytologic atypia and/or DCIS architecture within adenosis)

 Invasive Apocrine Carcinoma (Infiltrative growth, lacks myoepithelial layer)

 Sclerosing Adenosis without apocrine change (Lacks apocrine cytology)

Prognosis  Benign; risk similar to sclerosing adenosis

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Collagenous Spherulosis

Benign incidental finding with intraductal clusters of myoepithelial cells surrounding eosinophilic, acellular spherules of basement membrane material.

Clinical  Incidental microscopic finding, often within areas of sclerosing adenosis, papillomas, or other benign proliferative lesions

Radiology ─ May be associated with microcalcifications if occurring within other lesions

Macro  Not grossly visible

Micro ─

 Intraductal or intra-acinar lesion

 Clusters of bland myoepithelial-like cells arranged around eosinophilic, hyaline, acellular spherules

 Spherules represent basement membrane material (PAS+, Collagen IV+)

 Often forms cribriform-like pattern within the duct/acinus

 May have admixed epithelial cells

 No cytologic atypia or necrosis

IHC ─ Spindle cells surrounding spherules (+) myoepithelial markers (p63, SMA, calponin, SMMHC); Spherules (+) Collagen IV, Laminin; Luminal epithelial cells (if present) (+) CK7/8/18

DDx ─

 Cribriform DCIS (Low Grade) (Malignant epithelial cells, lacks myoepithelial cells within cribriform spaces, lacks eosinophilic spherules, lacks myoepithelial markers within spaces)

 Adenoid Cystic Carcinoma (Rare in breast; true cribriform spaces with basement membrane material, biphasic population, invasive)

Prognosis  Benign incidental finding; no associated increased risk

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Radial Scar / Complex Sclerosing Lesion (CSL)

Benign proliferative lesions with a central fibroelastotic core with entrapped ducts, from which ducts and lobules radiate outwards. Radial Scar < 1 cm, CSL ≥ 1 cm.

Clinical  Can occur at any age, more common > 40 years; often detected as mammographic abnormality (spiculated mass, distortion, calcifications), less often palpable

Radiology ─

 Mammography: Characteristically shows spiculated density with central radiolucency, architectural distortion; may have associated calcifications; mimics invasive carcinoma

 Ultrasound: Irregular hypoechoic mass with posterior shadowing

Macro  Ill-defined, firm, gray-white stellate lesion; central puckering may be visible

Micro ─

 Central fibroelastotic core: Hyalinized collagen and elastic fibers, often acellular or paucicellular, containing small, entrapped, often atrophic ducts/tubules

 Radiating ducts and lobules: Proliferative changes common in radiating elements, including cysts, UDH, apocrine metaplasia, sclerosing adenosis, columnar cell changes, +/- atypia (ADH/ALH) or DCIS

 Overall stellate architecture at low power

 Entrapped ducts within core retain myoepithelial layer

IHC ─ Myoepithelial markers useful to confirm benignity of entrapped ducts in core and distinguish from tubular carcinoma

DDx ─

 Tubular Carcinoma (Invasive angulated glands lacking myoepithelial layer, desmoplastic stroma, lacks central elastotic core and radiating benign structures)

 Invasive Carcinoma NOS with desmoplasia (Malignant cytology, lacks characteristic architecture)

 Sclerosing Adenosis (Lobulocentric, lacks central fibroelastotic core)

Prognosis  Benign lesion itself, but associated with slightly increased risk of subsequent breast cancer (~2x); risk higher if atypia (ADH/ALH) or DCIS is found within the lesion

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WSI DCIS involving radial sclerosing lesion

Lactating Adenoma

Benign tumor-like lesion occurring during pregnancy or lactation, composed of hyperplastic secretory lobules.

Clinical  Pregnant or lactating women (mc breast mass in this group); presents as palpable, mobile, often rapidly growing mass

Radiology ─

 Ultrasound: Typically circumscribed, hypoechoic or isoechoic mass, may be complex

Macro  Well-circumscribed, lobulated, soft, gray-white to tan mass

Micro ─

 Well-circumscribed, lobulated proliferation of glands resembling lactating breast tissue

 Acini markedly enlarged, lined by cuboidal cells with secretory changes (vacuolated cytoplasm, apical snouts, luminal secretions)

 Nuclei may be enlarged with prominent nucleoli (lactational change), but bland overall

 Minimal intervening stroma

 Mitotic figures may be present but are not atypical

 +/- Infarction (esp. post-partum)

IHC ─ Not typically required; epithelial cells (+) milk proteins (e.g., lactalbumin), ER/PR often negative during lactation

Molecular  Lacks MED12 mutations (unlike fibroadenoma)

DDx ─

 Fibroadenoma with lactational changes (Has characteristic fibroadenoma stroma, MED12 mutated)

 Secretory Carcinoma (Infiltrative growth, characteristic eosinophilic secretions, ETV6::NTRK3 fusion)

 Invasive Carcinoma with secretory features (Malignant cytology, infiltrative)

Prognosis  Benign; often regresses after cessation of lactation; excision may be performed for diagnosis or if persistent

Media  pathoutlines  WSI WSI WSI WSI WSI

Tubular Adenoma

Benign neoplasm of closely packed small tubules with minimal intervening stroma.

Clinical  young women; well-circumscribed, palpable mass

Radiology ─

 Mammography/Ultrasound: well-circumscribed, round or oval, may resemble fibroadenoma

Macro  Well-circumscribed, firm, tan-white nodule

Micro ─

 Sharply circumscribed or encapsulated

 Composed of tightly packed, small, uniform, round tubules

 Tubules lined by single layer of epithelial cells and outer layer of myoepithelial cells

 Epithelial cells cuboidal, bland nuclei, minimal cytoplasm

 Minimal intervening stroma

 +/- Apocrine metaplasia, calcifications

IHC ─ Epithelial cells (+) CK7/8/18, ER/PR; Myoepithelial layer (+) p63, SMA, etc.

DDx ─

 Fibroadenoma (Prominent stromal component, different architecture)

 Sclerosing Adenosis (Distorted/compressed glands, more stroma, lobulocentric)

 Tubular Carcinoma (Infiltrative, angulated glands, lacks myoepithelial layer, desmoplasia)

 Microglandular Adenosis (Haphazard glands, lacks myoepithelial layer, S100+)

Prognosis  Benign; excision is curative

Media  pathoutlines  WSI WSI WSI

Epithelial-myoepithelial tumors

Pleomorphic adenoma

Benign biphasic tumor composed of epithelial/ductal elements and myoepithelial cells within a mesenchymal (often chondromyxoid) stroma. Rare in the breast, identical to its salivary gland counterpart. Also known as benign mixed tumor.

Clinical  Adults (wide age range); well-circumscribed, painless, mobile mass

Radiology ─

 Mammography/Ultrasound: Typically shows well-circumscribed, lobulated mass; may have calcifications

Macro  Well-circumscribed, encapsulated, firm nodule; gray-white, glistening, cartilaginous cut surface

Micro ─

 Biphasic tumor with epithelial and myoepithelial components embedded in stroma

 Epithelial component: Ducts, tubules, or nests lined by cuboidal/columnar cells

 Myoepithelial component: Spindle, plasmacytoid, or clear cells, often blending with stroma

 Stromal component: Characteristically chondromyxoid (cartilage-like); may also be fibrous or hyalinized; osseous metaplasia possible

 Well-circumscribed, often encapsulated

 No significant atypia or mitoses

IHC ─

 Epithelial cells: (+) Keratins (CK7, CAM5.2), EMA

 Myoepithelial cells: (+) p63, CK5/6, S100, SMA, calponin, GFAP (variable)

 Stroma: (+) S100 if chondroid

Molecular  PLAG1 gene rearrangements (chromosome 8q12) common, often involving HMGA2 fusion

DDx ─

 Adenomyoepithelioma (Lacks chondromyxoid stroma, different architecture)

 Metaplastic Carcinoma (esp. low-grade adenosquamous or matrix-producing) (Infiltrative growth, cytologic atypia, malignant features)

 Phyllodes Tumor (Leaf-like architecture, lacks chondromyxoid stroma/true epithelial glands)

 Fibroadenoma with stromal metaplasia (Different stromal quality, lacks prominent myoepithelial component)

Prognosis  Benign; recurrence rare if completely excised; rare malignant transformation (carcinoma ex pleomorphic adenoma)

Media  pathoutlines   WSI

Adenomyoepithelioma

Benign biphasic tumor with proliferation of both epithelial (luminal) cells and myoepithelial cells, typically arranged in a tubular pattern, lacking significant stromal component.

Clinical  Adults (often older women); presents as palpable, well-circumscribed mass

Radiology ─

 Mammography/Ultrasound: Well-circumscribed, lobulated mass; may appear complex

Macro  Well-circumscribed, lobulated, firm, gray-white nodule

Micro ─

 Well-circumscribed, often lobulated proliferation

 Biphasic pattern with inner epithelial layer and outer myoepithelial layer

 Predominantly tubular architecture most common; solid or spindle cell patterns also occur

 Epithelial cells: Cuboidal to columnar, bland

 Myoepithelial cells: Often prominent, surrounding tubules; may be spindled or clear; typically bland

 Minimal intervening stroma (unlike pleomorphic adenoma)

 +/- Apocrine metaplasia, squamous metaplasia, CCH/ADH in adjacent tissue

 Mitoses rare, atypia minimal

IHC ─

 Epithelial cells: (+) Keratins (CK7, CAM5.2), EMA

 Myoepithelial cells: (+) p63, CK5/6, CK14, SMA, calponin, S100 (variable)

Molecular  Heterogeneous; PIK3CA, AKT1, HRAS, BRAF mutations reported; distinct from pleomorphic adenoma (PLAG1)

DDx ─

 Tubular Adenoma (Myoepithelial layer less prominent, lacks clear/spindle myoepithelial cells)

 Pleomorphic Adenoma (Has chondromyxoid stroma)

 Myoepithelioma (Pure myoepithelial proliferation, lacks distinct ductal component)

 Low-grade Adenosquamous Carcinoma / Syringomatous Tumor (Infiltrative growth, atypia)

 Tubular Carcinoma (Infiltrative, lacks myoepithelial layer)

 Malignant Adenomyoepithelioma / Adenomyoepithelioma with carcinoma (Malignant cytology in either component, infiltrative growth, necrosis, high mitoses)

Prognosis  Benign; low rate of local recurrence; rare malignant transformation

Media  pathoutlines  WSI WSI WSI WSI WSI  video  Malignant WSI WSI

Papillary Lesions and Neoplasia

Intraductal papilloma

Benign papillary neoplasm arising within a duct, composed of fibrovascular cores lined by epithelial and myoepithelial cells.

Clinical  Wide age range, mc 40-50s; often presents with nipple discharge (serous or bloody); may be palpable subareolar mass (central papilloma) or incidental finding (peripheral papilloma)

Radiology ─

 Mammography: May show solitary or multiple dilated ducts, well-circumscribed mass, or calcifications

 Ultrasound: Intraductal mass, cystic lesion with mural nodule, or dilated duct with internal echoes

 Ductography: Filling defect within a duct

Macro  Friable, tan-pink, papillary mass within a dilated duct lumen; often central/subareolar; peripheral papillomas are often smaller and multiple

Micro ─

 Intraductal proliferation with distinct fibrovascular cores

 Arborescent (branching) papillary fronds

 Lined by two cell layers: inner epithelial (cuboidal/columnar) and outer myoepithelial

 Myoepithelial layer must be present on stalks (key feature)

 Epithelial component often shows usual ductal hyperplasia (UDH), apocrine metaplasia, or squamous metaplasia

 Stroma may be fibrotic or hyalinized

 +/- Associated duct ectasia, hemorrhage, inflammation, calcifications

 Peripheral papillomas often arise in TDLUs, may show more sclerosis/adenosis features

IHC ─

 Epithelial cells: (+) CK7, ER (variable)

 Myoepithelial cells: (+) p63, CK5/6, SMA, calponin (highlighting presence on stalks)

 (-) High molecular weight keratins in epithelial cells (unlike UDH sometimes)

Molecular  Clonal; recurrent PIK3CA and AKT1 mutations common

DDx ─

 Usual Ductal Hyperplasia (UDH) (Solid or fenestrated growth, lacks true fibrovascular cores, streaming nuclei, mosaic CK5/6)

 Papillary DCIS (Lacks myoepithelial layer on stalks, significant cytologic atypia, monomorphic population)

 Encapsulated Papillary Carcinoma (Solid papillary carcinoma within fibrous capsule, lacks myoepithelial layer within papillae)

 Solid Papillary Carcinoma (Solid growth pattern, neuroendocrine features common, lacks myoepithelial layer within tumor)

 Invasive Papillary Carcinoma (Invasive growth pattern, lacks myoepithelial layer)

 Adenomyoepithelioma (Different architecture, prominent myoepithelial proliferation around tubules)

Prognosis  Benign; solitary central papillomas without atypia have minimal increased risk; multiple peripheral papillomas confer a slightly higher risk; risk increases significantly if associated with ADH or DCIS

Media  pathoutlines  HE ER CK5  Ultrasound  video 

WSI WSI + WSI & CK5/6 with UDH  WSI with ADH  WSI with DCIS

Papillary DCIS

Ductal carcinoma in situ (DCIS) with the formation of true papillae lined by neoplastic epithelial cells lacking a myoepithelial layer along the fibrovascular cores.

Clinical  Wide age range, often older women; may present with nipple discharge or as mammographic finding (calcifications, mass)

Radiology ─

 Mammography: May show calcifications, intraductal mass, or duct dilatation

 Ultrasound: May show intraductal mass or complex cystic lesion

Macro  Often involves large ducts; may form a friable mass within a duct or cyst

Micro ─

 Intraductal proliferation forming papillae with fibrovascular cores

 Papillae lined by neoplastic epithelial cells showing cytologic features of DCIS (usually low to intermediate grade atypia, but can be high grade)

 Key feature: Absence of myoepithelial cell layer along the fibrovascular cores of the papillae (must confirm with IHC)

 Myoepithelial layer is still present at the periphery of the involved duct

 May coexist with other patterns of DCIS (e.g., cribriform, solid)

 Necrosis may be present

IHC ─

 Epithelial cells: (+) ER, PR (usually); (+) CK7; (-) Myoepithelial markers (p63, CK5/6, SMA, etc.) on papillary stalks

 Peripheral myoepithelial layer: (+) Myoepithelial markers

Molecular  Similar genetic alterations to other forms of non-high grade DCIS

DDx ─

 Intraductal Papilloma (Benign; has myoepithelial layer on stalks)

 Intraductal Papilloma with ADH/DCIS (Atypical proliferation involves benign papilloma structure which retains focal myoepithelial cells on stalks)

 Encapsulated Papillary Carcinoma (Lacks peripheral duct structure/myoepithelial layer; surrounded by fibrous capsule)

 Solid Papillary Carcinoma (Predominantly solid growth, often neuroendocrine features, lacks true fibrovascular cores)

 Invasive Papillary Carcinoma (Invasive growth into stroma)

Prognosis  Considered DCIS; prognosis excellent with complete excision; risk of recurrence/progression similar to other DCIS subtypes of comparable grade

Media  pathoutlines  WSI   WSI   WSI

Solid Papillary Carcinoma (SPC)

Circumscribed papillary neoplasm with solid growth of monotonous, low-grade neuroendocrine-like epithelial cells arranged in nodules separated by delicate fibrovascular septa. Often considered a form of DCIS or low-grade invasive carcinoma depending on circumscription/invasion.

Clinical  Typically older women (postmenopausal); often presents as palpable mass or mammographic finding

Radiology ─

 Mammography/Ultrasound: Usually well-circumscribed, round or lobulated mass; may appear complex cystic/solid

Macro  Well-circumscribed, fleshy, tan-gray nodule; may have cystic areas

Micro ─

 Expansile, circumscribed nodules of solid epithelial proliferation

 Separated by delicate fibrovascular septa (true papillae with cores are rare/absent)

 Tumor cells: Monotonous, round to oval nuclei, often with neuroendocrine features (salt-and-pepper chromatin, inconspicuous nucleoli), eosinophilic/granular cytoplasm

 Solid growth pattern predominates; may have pseudorosettes or pseudoacini

 Myoepithelial cells are absent within the tumor nodules (present only at periphery if SPC is entirely intraductal/in situ)

 +/- Mucinous production (intracytoplasmic or extracellular)

 +/- Invasive component (usually low-grade invasive ductal carcinoma NOS) at periphery

IHC ─

 Tumor cells: (+) ER, PR (often strong); (+) Synaptophysin, Chromogranin (neuroendocrine markers, variable); (+) CK7; (-) Myoepithelial markers within nodules

 HER2 negative

Molecular  Frequent PIK3CA mutations

DDx ─

 Encapsulated Papillary Carcinoma (More prominent papillary/cribriform architecture, lacks neuroendocrine features)

 Papillary DCIS (True fibrovascular cores, lacks solid neuroendocrine-like growth)

 Intraductal Papilloma (Benign; myoepithelial layer present on stalks)

 Neuroendocrine Neoplasm (Metastatic or primary NET) (Different morphology, other neuroendocrine markers may be stronger, lacks ER/PR)

 Invasive Ductal Carcinoma NOS (If invasive component present, need to assess SPC features)

Prognosis  Generally indolent course; if entirely circumscribed (in situ SPC), prognosis excellent; risk increases if associated invasive carcinoma is present

Media  pathoutlines   WSI   WSI   WSI

Encapsulated Papillary Carcinoma (EPC)

Papillary carcinoma entirely enclosed within a fibrous capsule, lacking myoepithelial cells within the papillae and along the capsule boundary. Often considered a form of DCIS.

Clinical  Typically older women (postmenopausal); presents as palpable mass or mammographic finding

Radiology ─

 Mammography/Ultrasound: Well-circumscribed, round or oval mass, often complex cystic/solid

Macro  Well-circumscribed, often cystic nodule containing papillary structures; surrounded by fibrous capsule

Micro ─

 Papillary and/or cribriform proliferation of neoplastic epithelial cells

 Cells typically show low to intermediate grade atypia

 Contained entirely within a thick fibrous capsule

 Key feature: Absence of myoepithelial cell layer both within the papillary stalks and at the periphery/capsule interface (must confirm with IHC)

 True stromal invasion beyond the capsule is absent by definition

 +/- Hemorrhage, hemosiderin, foamy macrophages within cystic spaces

IHC ─

 Epithelial cells: (+) ER, PR (usually); (+) CK7; (-) Myoepithelial markers (p63, CK5/6, SMA, etc.) throughout the lesion including periphery

 HER2 negative

Molecular  Similar alterations to low/intermediate grade DCIS

DDx ─

 Intracystic Papilloma (Benign; has myoepithelial layer on stalks and/or periphery)

 Papillary DCIS (Contained within a native duct structure with peripheral myoepithelial cells; lacks thick fibrous capsule)

 Solid Papillary Carcinoma (Predominantly solid growth, neuroendocrine features)

 Invasive Papillary Carcinoma (Invasion beyond capsule into stroma)

 Invasive Cribriform Carcinoma (If predominantly cribriform; invasive component present)

Prognosis  Excellent prognosis, considered a form of non-invasive carcinoma (DCIS); risk of recurrence very low if completely excised

Media  pathoutlines  WSI   WSI           video

Lobular Neoplasia

Atypical Lobular Hyperplasia (ALH)

A neoplastic proliferation of small, monotonous, discohesive cells within terminal duct lobular units (TDLUs), partially filling and involving <50% of the acini within an individual lobule. Considered part of the spectrum of lobular neoplasia (LN).

Clinical  Primarily premenopausal women; usually an incidental microscopic finding on biopsy performed for other reasons (e.g., calcifications, fibrocystic changes)

Radiology ─

 No specific imaging features; often found in biopsies for calcifications which are usually related to adjacent benign changes (e.g., sclerosing adenosis)

Macro  Not grossly identifiable

Micro ─

 Proliferation of small, uniform, round cells with scant cytoplasm and indistinct borders

 Cells are discohesive, lacking cell-to-cell adhesion

 Involves acini of TDLUs, partially filling them; defined as involving <50% of the acini within a lobule

 Acini are generally not distended (unlike florid LCIS)

 Nuclei round to oval, often with inconspicuous nucleoli; minimal pleomorphism

 Intracytoplasmic lumina (mucin negative) may be present

 Pagetoid spread (single cells undermining ductal epithelium) into terminal ducts can occur

 Myoepithelial layer surrounding acini is intact

IHC ─

 (-) E-cadherin (membranous loss is characteristic)

 (+) p120 catenin (cytoplasmic staining pattern due to E-cadherin loss)

 (+) ER, PR

 (-) HER2

 (-) CK5/6, HMWK (negative in neoplastic cells)

Molecular

 Loss of CDH1 gene function (mutation or methylation)

 Loss of heterozygosity (LOH) at 16q (location of CDH1) common

DDx ─

 Lobular Carcinoma In Situ (LCIS) (Same cytology but involves 50% of acini in a lobule, often with distension)

 Ductal Carcinoma In Situ (DCIS), Low Grade (Cohesive cells, different architecture (cribriform, micropapillary), E-cadherin+)

 Usual Ductal Hyperplasia (UDH) (Polymorphous population, streaming/slit-like spaces, CK5/6 mosaic+, E-cadherin+)

 Benign lobules/Acinar cells in adenosis (Cohesive, maintain polarity, E-cadherin+)

Prognosis  Considered a risk factor (marker) for subsequent development of invasive carcinoma (relative risk ~4-5x) in both breasts; risk is lower than for LCIS

Media  pathoutlines   WSI   video video

Lobular Carcinoma In Situ (LCIS)

A neoplastic proliferation of small, monotonous, discohesive cells filling and distending ≥50% of the acini within a TDLU. Considered part of the spectrum of lobular neoplasia (LN).

Clinical  Primarily premenopausal women (mean age ~50); usually an incidental microscopic finding; not typically associated with a palpable mass or specific imaging findings, though may be near calcifications

Radiology ─

 No specific features; usually occult on imaging; may be found in biopsies for calcifications related to adjacent benign changes

Macro  Not grossly identifiable

Micro ─

 Proliferation of small, uniform, round cells with scant cytoplasm and indistinct borders

 Cells are discohesive, lacking cell-to-cell adhesion

 Fills and often distends most or all (50%) of the acini within affected TDLUs

 Nuclei round to oval, monotonous, often with inconspicuous nucleoli; minimal pleomorphism (Classic LCIS)

 Intracytoplasmic lumina (mucin negative) may be present

 Pagetoid spread (single cells undermining ductal epithelium) into terminal ducts and larger ducts is common

 Myoepithelial layer surrounding involved acini/ducts is intact

 Variants:

 Florid LCIS: Marked expansion of TDLUs by classic LCIS cells, often with central necrosis/calcification, forming confluent sheets

 Pleomorphic LCIS (PLCIS): Cells show greater nuclear pleomorphism (size 4x lymphocyte), larger nucleoli, more cytoplasm; often associated with necrosis/calcification; may mimic high-grade DCIS

IHC ─

 (-) E-cadherin (membranous loss is characteristic)

 (+) p120 catenin (cytoplasmic staining pattern)

 (+) ER, PR (usually strong/diffuse in classic LCIS; may be weaker or negative in PLCIS)

 (-) HER2 (may be amplified in PLCIS)

 (-) CK5/6, HMWK (negative in neoplastic cells)

Molecular

 Loss of CDH1 gene function (mutation or methylation)

 Loss of heterozygosity (LOH) at 16q (location of CDH1) common

 Complex alterations may be seen in PLCIS

DDx ─

 Atypical Lobular Hyperplasia (ALH) (Same cytology but involves <50% of acini within a lobule, minimal/no distension)

 Ductal Carcinoma In Situ (DCIS) (Cohesive cells, different architecture (cribriform, micropapillary, solid), E-cadherin+)

 Invasive Lobular Carcinoma (Infiltration into stroma, lacks surrounding myoepithelial layer)

 Lymphoma/Leukemia Infiltrate (LCA+, specific hematopoietic markers+)

 Benign lobules/Acinar cells in adenosis (Cohesive, maintain polarity, E-cadherin+)

Prognosis  Considered a risk factor (marker) and non-obligate precursor for subsequent invasive carcinoma (relative risk ~8-10x) in both breasts. Risk is higher than for ALH. Pleomorphic and florid LCIS may have higher risk and are often managed similarly to DCIS (excision with margins).

Media  pathoutlines   WSI video video case video

Pleomorphic pathoutlines  WSI   WSI

Florid   pathoutlines

Ductal Neoplasia

Atypical Ductal Hyperplasia (ADH)

A clonal intraductal proliferation of epithelial cells that resembles low-grade DCIS (architecturally and/or cytologically) but is quantitatively limited in extent (typically defined as involving ≤2 duct spaces or measuring ≤2 mm in linear extent).

Clinical  Usually an incidental microscopic finding, most often associated with mammographic calcifications biopsied for other reasons; peak age typically perimenopausal/postmenopausal

Radiology ─

 Mammography: Frequently associated with microcalcifications (amorphous, punctate, or pleomorphic), often grouped; may be seen with architectural distortion or densities but often has no specific correlate itself

Macro  Not grossly identifiable

Micro ─

 Proliferation of monotonous epithelial cells within ducts or TDLUs

 Cells resemble those of low-grade DCIS (round nuclei, mild atypia, distinct cell borders)

 Architectural patterns mimic low-grade DCIS:

 Cribriform: Rigid bridges, punched-out secondary lumens

 Micropapillary: Short, club-shaped projections without fibrovascular cores

 Solid: Ducts partially filled with monotonous cells

 Quantitative limitation is key for diagnosis: Involvement of only one or two duct spaces, AND/OR linear extent  2 mm

 Myoepithelial layer surrounding involved spaces is intact

IHC ─

 (+) ER, PR (usually strong and diffuse)

 (+) E-cadherin (membranous)

 (-) CK5/6 or HMWK (negative or only rare scattered positive cells within proliferation, unlike mosaic pattern in UDH)

 (-) HER2 (typically)

 (+) Myoepithelial markers (p63, etc.) highlight intact peripheral layer

Molecular

 Clonal proliferation; often shows LOH at 16q and 17p, similar to low-grade DCIS

DDx ─

 Usual Ductal Hyperplasia (UDH) (Heterogeneous population, streaming nuclei, slit-like spaces, mosaic CK5/6+, patchy ER)

 DCIS, Low Grade (Qualitatively similar but exceeds size criteria for ADH - >2mm or >2 ducts involved)

 Flat Epithelial Atypia (FEA) (Lacks complex architecture like cribriform/micropapillary patterns)

 LCIS involving ducts (Pagetoid spread) (Discohesive cells, E-cadherin negative)

Prognosis  Considered a high-risk lesion, conferring a significantly increased risk (relative risk ~4-5x) for subsequent development of invasive carcinoma in both breasts; often warrants excision biopsy if found on core needle biopsy due to risk of upgrade to DCIS or invasive carcinoma

Media  Pathoutlines

          WSI + stains  WSI WSI WSI            video   H&E   H&E     video

Ductal Carcinoma In Situ (DCIS)

A neoplastic proliferation of epithelial cells confined to the ductal-lobular system, with cytologic atypia and specific architectural patterns.

Clinical  Wide age range, more common with increasing age; detected as calcifications; less commonly as palpable mass, discharge, or Paget disease of nipple

Radiology ─

 Mammography: Microcalcifications (pleomorphic, linear, branching, casting) are the most common finding; may also present as mass, density, or architectural distortion

 Ultrasound: May show duct dilatation, intraductal mass, or non-specific findings; less sensitive than mammography, especially for calcification-only DCIS

 MRI: May show non-mass enhancement or clumped enhancement

Macro  Often grossly occult; may appear as firm, gray-white areas, dilated ducts with cheesy material (comedo type), or cystic lesion (papillary types)

Micro ─

 Proliferation of neoplastic epithelial cells confined within ducts and/or lobules (cancerization of lobules)

 Myoepithelial cell layer is present around involved ducts/lobules (though may be attenuated)

 Cytologic atypia present, ranging from low to high grade

 Various architectural patterns (often mixed):

 Comedo: High-grade nuclei, central necrosis (often calcified), solid sheets peripherally

 Cribriform: Rigid, punched-out, round secondary lumens within solid proliferation

 Micropapillary: Finger-like projections lacking fibrovascular cores, attached to duct wall

 Papillary: True fibrovascular cores lined by neoplastic cells, lacking myoepithelial layer on stalks (see Papillary DCIS outline)

 Solid: Ducts completely filled by neoplastic cells

 Flat: Resembles FEA but with higher grade nuclei or more extensive involvement (less common pattern)

 Grading (typically 3-tiered: Low, Intermediate, High): Based primarily on nuclear pleomorphism and presence/absence of comedonecrosis

 Low Grade: Monotonous nuclei (1.5-2x RBC), inconspicuous nucleoli, rare mitoses, necrosis usually absent (includes cribriform, micropapillary patterns)

 Intermediate Grade: Moderate pleomorphism, visible nucleoli, +/- necrosis, moderate mitoses

 High Grade: Marked pleomorphism (>2.5-3x RBC), prominent nucleoli, frequent mitoses, comedonecrosis characteristic

IHC ─

 (+) ER, PR (Common in low/intermediate grade, often negative in high grade/comedo)

 (+) E-cadherin (membranous, confirms ductal lineage)

 (+) CK7, CAM5.2 (epithelial cells)

 (-) Myoepithelial markers within neoplastic proliferation (e.g., p63, CK5/6, SMA); highlights intact peripheral myoepithelial layer

 HER2 overexpression/amplification common in high-grade/comedo DCIS, rare in low grade

Molecular

 Clonal proliferation with genetic alterations similar to invasive ductal carcinoma of corresponding grade

 Low grade: Often 16q loss, 1q gain

 High grade: More complex alterations, TP53 mutations, MYC amplification, HER2 amplification common

DDx ─

 Atypical Ductal Hyperplasia (ADH) (Similar cytology/architecture to low-grade DCIS but quantitatively limited - typically <2 ducts involved or <2mm extent)

 Usual Ductal Hyperplasia (UDH) (Heterogeneous population, streaming, slit-like spaces, mosaic CK5/6)

 Flat Epithelial Atypia (FEA) (Less atypia, lacks complex architecture)

 LCIS with ductal spread (Discohesive cells, E-cadherin negative)

 Intraductal Papilloma (Benign; myoepithelial layer present on stalks)

 Invasive Carcinoma (Breach of basement membrane/myoepithelial layer, stromal invasion)

Prognosis  Non-invasive precursor lesion; excellent prognosis with complete excision; main risk is local recurrence (as DCIS or invasive carcinoma) or development of new primary in either breast; risk of recurrence influenced by grade, size, margin status

Media  WSI Low grade cribriform

Invasive breast carcinoma

Microinvasive carcinoma

Invasive carcinoma ≤ 1 mm in greatest dimension.

Clinical  Detected due to associated DCIS

Micro ─

 Focus or foci of invasion measuring  1 mm in greatest dimension

 Infiltration of neoplastic cells into stroma beyond basement membrane/myoepithelial layer

 Single cells, small clusters, or tiny glands

DDx ─

 DCIS extending into sclerosing adenosis/radial scar (Retains myoepithelial layer)

 Extensive DCIS with tangential sectioning mimicking invasion

 True invasive carcinoma > 1 mm

Prognosis  Excellent prognosis, similar to DCIS alone, guidelines for DCIS

Media  pathoutlines

Invasive lobular carcinoma (ILC)

Invasive carcinoma of small, discohesive cells, often infiltrating in single-file strands or targetoid patterns, associated with loss of E-cadherin.

Clinical  Often postmenopausal; ill-defined mass; higher incidence of bilaterality

Radiology ─

 Mammography: ill-defined density, distortion, asymmetry; calcifications less common

 Ultrasound: ill-defined hypoechoic mass with posterior shadowing or no discrete lesion

 MRI: non-mass enhancement; more sensitive for detecting extent and multifocality

Macro  may lack a discrete mass

Micro ─

 Infiltrative proliferation of small, relatively uniform, discohesive cells

 Cells round to oval with scant cytoplasm, round nuclei, inconspicuous nucleoli (classic)

 Single-file strands ("Indian files") infiltrating stroma, minimal desmoplasia

 Targetoid pattern (concentric rings around benign ducts or lobules)

 Intracytoplasmic lumina (mucin negative) common

 Signet-ring cell features (intracytoplasmic mucin) may be present

 Minimal tubule formation

 Variants: Classic, solid, alveolar, pleomorphic (larger cells, more atypia/mitoses)

IHC ─

 (-) E-cadherin (membranous loss)

 (+) p120 catenin (cytoplasmic)

 (+) ER, PR

 (-) HER2 (amplification can occur in pleomorphic variant)

 (+) CK7; (-) CK5/6

Molecular

 Loss of CDH1

 PIK3CA, TBX3, FOXA1 mutations common

 ERBB2 (HER2) amplification in pleomorphic variant

DDx ─

 Invasive Ductal Carcinoma NOS (Cohesive, tubule formation, E-cadherin+)

 DCIS or LCIS with infiltration artifact (myoepithelial layer around in situ component)

 Lymphoma/Leukemia

 Metastatic carcinoma (esp. gastric signet ring)

Prognosis  ~IDC NOS; Pleomorphic worse than classic.

Media  WSI   WSI  classic WSI   solid & pleomorphic WSI     HE pleomorphic  HE Histiocytoid  HE solid  p120

Tubular Carcinoma

Well-differentiated invasive carcinoma composed predominantly (≥90%) of single-layered tubules infiltrating stroma; low-grade malignancy.

Clinical  Often perimenopausal/older women; frequently detected mammographically as small spiculated density (1 cm), may have microcalcifications; can be palpable.

Radiology ─

 Mammography: Small (<2cm), irregular or spiculated mass; often associated microcalcifications.

 Ultrasound: Hypoechoic, irregular mass with posterior acoustic shadowing.

 MRI: Irregular enhancing mass, useful for extent if needed.

Macro  Small (often 1 cm), firm, gritty, irregular/stellate borders; may be difficult to identify grossly.

Micro ─

 Infiltrating, well-formed, open tubules; haphazardly arranged, often angulated/teardrop-shaped.

 Key: Tubules lined by a single layer of epithelial cells (no myoepithelial layer).

 Epithelial cells: Small, uniform, cuboidal; low-grade nuclei (round, regular, inconspicuous nucleoli, rare mitoses).

 Apical snouts (decapitation secretion) characteristic and prominent.

 Minimal cytologic atypia or pleomorphism.

 Stroma: Desmoplastic/fibroelastotic.

 Purity: 90% tubular pattern required for diagnosis; <90% is IDC NST with tubular features.

 Often associated with low-grade DCIS, columnar cell lesions, LCIS (Rosen Triad).

IHC ─

 (+) ER, PR

 (-) HER2

 (+) E-cadherin (Membranous)

 (-) Myoepithelial markers (p63, SMA, Calponin, SMMHC) around invasive tubules.

 (-) Basal Cytokeratins (CK5/6)

Molecular  Consistent with Luminal A subtype (ER+/PR+/HER2-). Specific recurrent mutations less defining than IHC profile.

DDx ─

 Sclerosing Adenosis: Benign; compressed glands retain myoepithelial layer (IHC+).

 Microglandular Adenosis: Benign; round glands lack myoepithelial layer but have basement membrane; ER/PR(-), S100(+).

 Radial Scar/Complex Sclerosing Lesion: Benign; entrapped glands retain myoepithelial layer.

 Invasive Cribriform Carcinoma: Well-differentiated; distinct cribriform architecture.

 Tubulolobular Carcinoma: Features of both tubular and lobular carcinoma.

Prognosis  Excellent; significantly better than IDC NST. Low rate of lymph node metastasis (~10-15%); distant metastasis rare.

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Tubulolobular Carcinoma

Invasive carcinoma showing mixed features of both tubular carcinoma (tubules) and classic invasive lobular carcinoma (single file cells)

Clinical  Similar to tubular or lobular carcinoma; often small irregular mass on mammography

Radiology ─

 Mammography: Small irregular or spiculated mass; possibly calcifications

 Ultrasound: Irregular hypoechoic mass; possibly shadowing

 MRI: Mixed features; useful for determining extent

Macro  Small (less than 2 cm), firm, gray-white, infiltrative borders

Micro ─

 Mixture: infiltrating tubules (single layer, angulated, apical snouts) plus infiltrating single file strands or discohesive cells

 Absent myoepithelial cells surrounding tubules

 Cells are small, uniform, low-grade nuclei (similar to classic lobular and tubular carcinoma)

 Intracytoplasmic lumina possible

 Minimal stromal reaction surrounding single files; variable surrounding tubules

 Tubular and lobular mixture varies

IHC ─

 (+) E-cadherin (Membranous; key difference from classic lobular carcinoma)

 (+) p120 catenin (Membranous)

 (+) ER, PR

 (-) HER2

 (-) Myoepithelial markers (p63, SMA, Calponin) surrounding invasive components

Molecular

 Absent CDH1 loss (unlike classic lobular carcinoma)

 Luminal A subtype (ER/PR+/HER2 negative)

DDx ─

 Tubular Carcinoma: Greater than 90% tubules; no significant single file component

 Classic Lobular Carcinoma: Mostly single file cells; no tubules; E-cadherin negative

 Invasive Ductal Carcinoma NST with Tubular and Lobular Features: Does not meet strict criteria; E-cadherin+

 Invasive Cribriform Carcinoma: Cribriform architecture; E-cadherin+

Prognosis  Favorable; better than classic lobular carcinoma and invasive ductal carcinoma NST; possibly slightly worse than pure tubular carcinoma; depends on mixture and grade; low lymph node metastasis

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Invasive Cribriform Carcinoma

Invasive carcinoma with cribriform growth pattern similar to cribriform DCIS

Clinical  Similar presentation to invasive carcinoma NST

Radiology ─ Nonspecific mass or calcifications

Macro  Nonspecific firm mass

Micro ─

 Infiltrating nests of cells with fenestrated rounded or angulated gland-like spaces (cribriform pattern)

 Tumor cells usually low to intermediate nuclear grade

 Mucinous secretion or microcalcifications sometimes present in lumens

 Stroma often fibroblastic

 Pure form has greater than 90% cribriform morphology

 Tubular growth pattern commonly seen as minor component (less than 50%)

 Other patterns present denote mixed type

IHC ─

 (+) ER, PR

 (-) HER2

 (+) E-cadherin

 (-) Myoepithelial markers around invasive nests

Molecular  Consistent with Luminal A subtype ((+)ER/PR positive/HER2 negative)

DDx ─

 Cribriform DCIS: Retains myoepithelial layer (check with IHC)

 Adenoid Cystic Carcinoma: Biphasic (epithelial/myoepithelial); distinct pseudolumens with basal lamina material; often CD117 positive

 Tubular Carcinoma: Predominantly well-formed single-layered tubules; lacks cribriform pattern

 Invasive Carcinoma NST with Cribriform Features: Less than 90% cribriform pattern

Prognosis  Favorable; better than invasive carcinoma NST

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Mucinous Carcinoma

Invasive carcinoma with extracellular mucin production

Clinical  Typically affects older postmenopausal women; often presents as palpable mass

Radiology ─

 Mammography: circumscribed, lobulated; isodense or hyperdense; calcification uncommon

 Ultrasound: complex cystic and solid mass; may be isoechoic or hypoechoic

 MRI: Variable appearance; often high signal on T2-weighted images

Macro  Well-circumscribed gelatinous or mucoid mass; gray-blue cut surface

Micro ─

 Nests clusters or islands of tumor cells floating in abundant extracellular mucin pools

 Tumor cells typically small uniform low-grade nuclei; may show neuroendocrine features

 Pure mucinous carcinoma: Greater than 90% mucinous pattern

 Mixed mucinous carcinoma: 1090% mucinous pattern admixed with non-mucinous invasive carcinoma (usually NST)

 Micropapillary variant: Small clusters of cells with reversed polarity within mucin pools; more aggressive behavior

 Cellular variant: More cellular nests with less abundant mucin

 Signet-ring cells may be present

IHC ─

 (+) ER, PR

 (-) HER2

 (+) E-cadherin

 (+) Chromogranin/Synaptophysin

 Mucin stains (Alcian blue PAS mucicarmine) positive for extracellular mucin

Molecular

 Consistent with Luminal A subtype ((+)ER/PR positive/HER2 negative)

 Lower frequency of TP53 mutations compared to invasive carcinoma NST

 Specific molecular alterations not well-defined for pure type

DDx ─

 Mucocele-like lesion: Benign; lacks atypia; often associated with ADH or DCIS; extravasated mucin lacks floating epithelial cells

 Invasive Carcinoma NST with Mucin Production: Less than 90% mucinous pattern

 Metastatic Mucinous Carcinoma (e g GI tract ovary): Check clinical history; use organ-specific IHC markers (e g CDX2 SATB2 PAX8 CK20)

 Signet Ring Cell Carcinoma (Lobular variant): Predominantly intracellular mucin; E-cadherin negative

Prognosis  Pure mucinous carcinoma generally has a favorable prognosis; better than invasive carcinoma NST; low rate of lymph node metastasis Mixed type has prognosis similar to the non-mucinous component Micropapillary variant is more aggressive

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Invasive Carcinoma with Medullary Features

Poorly differentiated carcinoma with prominent lymphoplasmacytic infiltrate syncytial growth and pushing margins (Note: True "Medullary Carcinoma" is no longer a distinct WHO category; now termed "Invasive Carcinoma with Medullary Features")

Clinical  Younger age group often; may be associated with BRCA1 mutation; presents as palpable mass

Radiology ─ Typically well-circumscribed round or oval mass; may mimic benign lesion like fibroadenoma; calcifications uncommon

Macro  Well-circumscribed soft fleshy gray-white mass; may show hemorrhage or necrosis

Micro ─

 Syncytial growth pattern (>75%): Broad sheets of cells with indistinct borders

 High nuclear grade: Large pleomorphic nuclei vesicular chromatin prominent nucleoli

 High mitotic rate often atypical forms

 Prominent diffuse lymphoplasmacytic infiltrate within and surrounding tumor nests

 Pushing non-infiltrative microscopic border

 No glandular differentiation

 Minimal associated DCIS if present

IHC ─

 (-) ER, PR, HER2

 (+) Basal cytokeratins (CK5/6 CK14 CK17)

 (+) EGFR

 (+) p53 due to mutation

Molecular

 Frequently associated with BRCA1 germline mutations

 Basal-like molecular subtype by gene expression profiling

 High frequency of TP53 mutations

DDx ─

 Poorly Differentiated Invasive Carcinoma NST: Lacks the combination of syncytial growth diffuse prominent lymphoid infiltrate and pushing borders

 Metastatic Carcinoma (e g melanoma lymphoma): Clinical history and specific IHC markers needed

 Lymphoma

Prognosis  Historically considered better than typical high-grade invasive carcinoma NST when strict criteria met; however prognosis is similar to other high-grade triple-negative/basal-like carcinomas when matched for stage

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Papillary Carcinoma (Invasive)

Invasive carcinoma forming papillae lined by neoplastic cells (Note: Includes several entities like invasive solid papillary carcinoma invasive papillary carcinoma NST invasive encapsulated papillary carcinoma)

Clinical  Often older women; may present as mass or nipple discharge

Radiology ─ Well-circumscribed or complex cystic/solid mass; may have calcifications

Macro  Often well-circumscribed; may be solid or cystic with papillary projections

Micro ─

 Invasive component shows papillary architecture: Fibrovascular cores lined by neoplastic epithelium invading stroma

 Neoplastic cells typically low to intermediate grade; may show neuroendocrine features (especially in solid papillary carcinoma)

 Encapsulated Papillary Carcinoma (EPC): Papillary proliferation within a cyst-like space surrounded by thick fibrous capsule; lacks myoepithelial cells at periphery; invasion often occurs beyond capsule as tubular carcinoma or IDC NST

 Solid Papillary Carcinoma (SPC): Expansile solid nodules of monotonous cells arranged around delicate fibrovascular cores; often neuroendocrine differentiation; invasion often associated usually low grade

 Invasive Papillary Carcinoma NST: True fibrovascular cores lined by malignant cells directly invading stroma; rare

IHC ─

 (+) ER, PR

 (-) HER2

 (+) Neuroendocrine markers in SPC

 (-) Myoepithelial markers absent around invasive papillae and at periphery of EPC/SPC invasion fronts

Molecular

 Generally Luminal type

 PIK3CA mutations common in some papillary lesions

DDx ─

 Intraductal Papilloma: Benign; has myoepithelial layer around papillae and periphery

 Intraductal Papillary Carcinoma (DCIS): Confined to ducts; myoepithelial layer present at periphery

 Invasive Micropapillary Carcinoma: Lacks true fibrovascular cores; distinct morphology and IHC profile

 Metastatic Papillary Carcinoma (e g ovary thyroid): Clinical history and organ-specific IHC needed

Prognosis  Generally favorable especially for invasive SPC and EPC when invasion is low grade/limited; Invasive papillary carcinoma NST prognosis depends on grade/stage

Invasive Micropapillary Carcinoma

Invasive carcinoma with small hollow clusters (morules) of neoplastic cells lying within clear stromal spaces resembling dilated lymphatics

Clinical  Similar age and presentation to invasive carcinoma NST; may be multifocal

Radiology ─ Often spiculated mass; frequently associated calcifications; may show extensive lymphadenopathy

Macro  No specific features; often ill-defined firm mass

Micro ─

 Small tight clusters or morules of neoplastic cells arranged around a central lumen or as solid balls

 Clusters situated within clear empty stromal spaces that mimic vascular channels

 Characteristic "inside-out" growth pattern: Cells at periphery of clusters show reversed polarity (apical surface faces stroma)

 Cells usually intermediate to high nuclear grade

 Frequent lymphovascular invasion often extensive

 Pure form (>90% micropapillary pattern) is rare; usually mixed with invasive carcinoma NST or other types

IHC ─

 (+) ER, PR

 (+) HER2 (~40-70%)

 (+) EMA shows distinct "inside-out" staining pattern (membranous staining on stromal-facing/peripheral surface of cell clusters)

 (-) CD34/D2-40 negative staining of stromal spaces confirms they are not true vascular channels (pseudo-lymphovascular spaces)

Molecular

 Often Luminal B molecular subtype ((+)ER/HER2) or HER2-enriched subtype

 TP53 mutations common

DDx ─

 Lymphovascular Invasion by other Carcinoma types: True endothelial lining (CD34/D2-40+); tumor cells lack reversed polarity and characteristic morule formation

 Mucinous Carcinoma Micropapillary Variant: Tumor clusters float in extracellular mucin pools not clear stromal spaces

Prognosis  Generally considered aggressive; high frequency of lymph node metastasis even with small primary tumor size; worse prognosis compared to invasive carcinoma NST when matched for stage

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Invasive Carcinoma with Apocrine Differentiation

Invasive carcinoma no special type showing focal apocrine features (typically >10% of tumor cells)

Clinical  Similar age and presentation to invasive carcinoma no special type

Radiology ─ Nonspecific findings similar to invasive carcinoma no special type

Macro  Nonspecific features similar to invasive carcinoma no special type

Micro ─

 Invasive carcinoma no special type architecture (glands nests cords sheets)

 Focal population (>10%) of cells with apocrine morphology: abundant eosinophilic granular cytoplasm large nuclei prominent nucleoli

 Nuclear grade often intermediate to high in apocrine areas

 Background carcinoma is typically invasive carcinoma no special type

IHC ─

 Apocrine areas: (-) ER PR (+) AR (+) GCDFP-15

 Non-apocrine areas: Variable ER PR HER2 status similar to invasive carcinoma no special type overall

 HER2 status can be positive or negative

Molecular

 Reflects the underlying invasive carcinoma no special type component

 Apocrine component often shows molecular features similar to pure apocrine carcinoma (e g PIK3CA/AKT/mTOR pathway alterations)

DDx ─

 Apocrine Carcinoma (Pure): >90% of tumor shows apocrine morphology

 Invasive Carcinoma NST: Lacks significant apocrine features (<10%)

 Benign Apocrine Metaplasia/Adenosis: Lacks invasion; retains myoepithelial layer (may be attenuated); lacks significant atypia

Prognosis  Generally determined by the grade stage and biomarker status of the overall tumor (invasive carcinoma no special type component); significance of focal apocrine differentiation itself is debated

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Apocrine Carcinoma

Invasive carcinoma composed almost exclusively (>90%) of cells with apocrine differentiation

Clinical  Similar age and presentation to invasive carcinoma no special type; may occur in older women

Radiology ─ Nonspecific mass often circumscribed or irregular; calcifications may be present

Macro  Nonspecific firm mass gray-white tan cut surface

Micro ─

 Infiltrating cells with distinct apocrine morphology: abundant eosinophilic granular cytoplasm large round nuclei prominent eosinophilic nucleoli distinct cell borders

 Architectural patterns variable: solid nests tubules trabeculae sometimes papillary

 Nuclear grade usually intermediate to high (grade 2 or 3)

 Mitotic figures often present

 May be associated with apocrine ductal carcinoma in situ

IHC ─

 (-) ER, PR (or weak/focal)

 (+) AR

 (+) GCDFP-15

 (+) HER2 (~30-50%)

 (+) CK7 CK8/18

 (-) CK5/6

Molecular

 Often HER2 enriched or molecular apocrine subtype

 Frequent PIK3CA AKT1 and TP53 mutations

 HER2 (ERBB2) amplification common

 Androgen receptor pathway activation

DDx ─

 Invasive Carcinoma with Apocrine Differentiation: <90% apocrine morphology

 Oncocytic Carcinoma: Cells packed with mitochondria (ultrastructure or specific stains); lacks prominent nucleoli of apocrine carcinoma; AR negative

 Histiocytoid Carcinoma: Variant of lobular carcinoma; E-cadherin negative; cells resemble histiocytes

 Granular Cell Tumor: Benign neural tumor; S100+; lacks true glandular differentiation

 Metastatic Carcinoma (e g kidney liver): Clinical history and organ-specific markers needed

Prognosis  Controversial; often considered similar to invasive carcinoma no special type when matched for grade and stage; HER2 status is a key prognostic/predictive factor

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Metaplastic Carcinoma

Heterogeneous group of carcinomas showing differentiation towards squamous cells spindle cells mesenchymal elements (chondroid osseous) or a mixture

Clinical  Wide age range often presents as large palpable rapidly growing mass; less frequent lymph node metastasis compared to invasive carcinoma no special type but higher risk of distant metastasis

Radiology ─ Often well-circumscribed dense mass on mammography; may appear cystic on ultrasound; MRI features variable

Macro  Typically well-circumscribed or lobulated firm mass; may have cystic areas hemorrhage or necrosis; cut surface can be fleshy gray-white or show cartilaginous/bony areas

Micro ─ Highly variable depending on subtype:

 Squamous Cell Carcinoma: Pure squamous differentiation or mixed with adenocarcinoma; keratinization intercellular bridges

 Spindle Cell (Sarcomatoid) Carcinoma: Predominantly atypical spindle cells often in fascicles; may have epithelioid areas; minimal glandular component

 Carcinoma with Mesenchymal Differentiation (Carcinosarcoma): Admixture of carcinomatous (ductal squamous or spindle) and malignant mesenchymal elements (chondrosarcoma osteosarcoma rhabdomyosarcoma fibrosarcoma)

 Matrix-Producing Carcinoma: Carcinoma cells within an abundant chondromyxoid matrix

 Low-Grade Adenosquamous Carcinoma: Infiltrating small glands and solid squamous nests in a fibrous/spindle cell stroma; low-grade cytology

 Fibromatosis-like Metaplastic Carcinoma: Bland spindle cells infiltrating in long fascicles mimicking fibromatosis; low-grade cytology

 Associated ductal carcinoma in situ may be present but often minimal or absent

IHC ─

 (-) ER, PR, HER2 (Typically triple-negative >90%)

 (+) Cytokeratins (variable often including high-molecular-weight CK5/6 34betaE12 CK14) confirms epithelial nature even in spindle/mesenchymal areas; positivity may be focal

 (+) p63/p40 (squamous and spindle cell)

 (+) EGFR

 Mesenchymal markers (S100 desmin SMA) may be positive in areas with corresponding differentiation

Molecular

 Predominantly basal-like or claudin-low molecular subtypes

 High frequency of TP53 mutations

 Frequent alterations in Wnt and PI3K pathways

 Epithelial-mesenchymal transition (EMT) pathway activation common

DDx ─

 Phyllodes Tumor (Malignant): Biphasic tumor with benign epithelial component and malignant stroma; lacks diffuse cytokeratin positivity in stroma; CD34 often positive in stroma

 Primary Sarcoma (e g angiosarcoma fibrosarcoma): Exceedingly rare; lacks epithelial component/cytokeratin positivity

 Fibromatosis: Bland spindle cells; nuclear beta-catenin positivity; lacks cytokeratin positivity

 Nodular Fasciitis: Reactive spindle cell proliferation; lacks cytokeratin positivity

 Invasive Carcinoma NST with Spindle Cell Change: Focal spindle cells within conventional adenocarcinoma

Prognosis  Generally poor compared to invasive carcinoma no special type; prognosis varies by subtype (low-grade variants better); high risk of hematogenous metastasis (lung brain) despite lower rate of nodal involvement

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Rare and salivary gland-type tumors

Acinic Cell Carcinoma

Rare invasive carcinoma showing serous acinar differentiation

Clinical  Nonspecific

Radiology ─ Nonspecific, often well-circumscribed

Macro  Well-circumscribed mass

Micro ─

 Infiltrating cells in solid sheets microcystic acinar or glandular patterns

 Cells have abundant granular cytoplasm due to zymogen-like granules

 Nuclei round, uniform, eccentrically located; nucleoli inconspicuous

 Mitotic activity low

IHC ─

 (+)Cytokeratins (CK7 CK8/18)

 (+)Amylase, Lysozyme, Chymotrypsin (acinar differentiation)

 (+)S100, DOG1

 (-)ER, PR, HER2

Molecular

 None, does not share the genomic rearrangement of salivary ACC

DDx ─

 Secretory Carcinoma: intracellular and extracellular secretions; ETV6::NTRK3

 Apocrine Carcinoma: eosinophilic granular cytoplasm but prominent nucleoli; (-)ER/PR (+)AR, (+)GCDFP-15

 Mucinous Carcinoma: Extracellular mucin pools

 Metaplastic Carcinoma: Higher grade and associated with other metaplastic elements

 Microglandular Adenosis: lacks atypia; (+)S100 but (-)ER/PR negative

Prognosis  indolent with favorable prognosis

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Adenoid Cystic Carcinoma

Invasive carcinoma morphologically identical to adenoid cystic carcinoma of salivary glands with dual epithelial and myoepithelial cell population

Micro ─

 Infiltrating nests and islands with characteristic patterns

 Cribriform: pseudolumens with eosinophilic hyaline or basophilic mucoid material

 Tubular: small tubules lined by inner epithelial and outer myoepithelial cells

 Solid: sheets of basaloid cells with minimal gland formation (worse prognosis)

 Two cell types: Small basaloid myoepithelial cells, larger epithelial cells lining true lumens

 Perineural invasion common and characteristic

IHC ─

 Epithelial cells: (+) CK7 EMA CD117

 Myoepithelial cells: (+) p63 p40 SMA Calponin S100

 Basal lamina material in pseudolumens: (+) Collagen IV

 (-)ER, PR, HER2

Molecular

 MYB::NFIB (similar to salivary gland)

DDx ─

 Invasive Cribriform Carcinoma: Lacks biphasic population; lacks basement membrane material in lumens; (+)ER; lacks MYB fusion

 Collagenous Spherulosis: myoepithelial cells surrounding eosinophilic spherules

 Solid Papillary Carcinoma: Neuroendocrine features common; (+)ER; not biphasic

 Metastatic Adenoid Cystic Carcinoma

Prognosis  indolent, low metastatic potential; late recurrence; solid pattern worse

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Secretory Carcinoma

Rare carcinoma with abundant intracellular and extracellular eosinophilic secretions forming vacuoles and microcysts; previously known as juvenile secretory carcinoma

Clinical  Historically described in children/adolescents but occurs across all ages including adults; usually presents as well-defined palpable mass

Radiology ─ Typically well-circumscribed mass; may be complex cystic/solid

Macro  Well-circumscribed firm tan-white or gray mass; may have cystic areas

Micro ─

 Infiltrating tumor cells arranged in solid microcystic glandular and sometimes papillary patterns

 Abundant intracellular and extracellular eosinophilic secretions resembling thyroid colloid or milk

 Secretions (+)PASD

 Cells are generally bland with low-grade vesicular nuclei small nucleoli and vacuolated cytoplasm

 Mitotic figures rare

IHC ─

 (+) S100

 (+) CK7 EMA Mammaglobin GCDFP-15 (variable)

 (+) E-cadherin

 (-) ER, PR, HER2

 (-) Myoepithelial markers around invasive component

Molecular

 ETV6::NTRK3 gene fusion resulting from t(12;15) translocation (same fusion found in infantile fibrosarcoma and congenital mesoblastic nephroma)

DDx ─

 Mucinous Carcinoma: Extracellular mucin is pale basophilic not eosinophilic granular; lacks ETV6 fusion

 Acinic Cell Carcinoma: Granular cytoplasm; lacks prominent secretions/vacuoles

 Lipid-Rich Carcinoma: Cytoplasmic lipid vacuoles; lacks eosinophilic secretions

 Glycogen-Rich Clear Cell Carcinoma: Abundant clear cytoplasm due to glycogen (PAS+ diastase sensitive); lacks eosinophilic secretions

 Apocrine Carcinoma: Granular eosinophilic cytoplasm prominent nucleoli; AR+

Prognosis  Generally excellent prognosis especially in children; considered indolent with very low metastatic potential although recurrences and rare metastases can occur particularly in adults

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Mucoepidermoid Carcinoma

Invasive carcinoma composed of a mixture of mucin-producing, squamous, and intermediate cells, similar to salivary gland mucoepidermoid carcinoma

Clinical  Rare, wide age range, nonspecific presentation usually palpable mass

Radiology ─ Nonspecific mass, may be cystic or solid

Macro  Variable, often cystic or solid mass, gray-white cut surface

Micro ─

 Triad of cell types: mucin-producing cells (glandular or signet ring), squamous cells (may show keratinization), and intermediate cells (undifferentiated)

 Cells arranged in solid nests, cysts, and glandular structures

 Variable amounts of extracellular and intracellular mucin

 Stroma often fibrous, may have prominent lymphoid infiltrate

 Classified into low, intermediate, and high grades based on proportion of cystic structures, neural invasion, necrosis, anaplasia, and mitotic rate (similar to salivary gland grading)

IHC ─

 Epithelial cells: (+) Cytokeratins (CK7, CK8/18, CK5/6, CK14, p63, p40 especially in squamous/intermediate cells)

 Mucin stains (+) (PAS, Alcian blue, mucicarmine)

 (-) ER, PR, HER2 (Typically triple-negative)

 (-) S100 protein, SMA (helps distinguish from some salivary gland type tumors)

Molecular

 Characteristic MAML2 gene rearrangement (resulting from t(11;19) translocation) found in a subset of cases, similar to salivary gland counterpart

 Often basal-like phenotype

DDx ─

 Metaplastic Carcinoma with Squamous Differentiation: Lacks significant mucinous or intermediate cell component; may have spindle/mesenchymal elements

 Adenoid Cystic Carcinoma: Biphasic population, distinct cribriform pattern with basement membrane material; (+) CD117; MYB fusion common

 Secretory Carcinoma: Characteristic secretions; ETV6::NTRK3 fusion common

 Metastatic Mucoepidermoid Carcinoma: Check clinical history for primary elsewhere (salivary gland, lung)

Prognosis  Variable, depends on histologic grade; low-grade tumors generally have favorable prognosis, high-grade tumors are more aggressive with potential for metastasis

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Tall Cell Carcinoma with Reversed Polarity (Pattern)

(Note: Not a distinct WHO entity, but a specific architectural and cytological pattern, most commonly seen as a component of Invasive Micropapillary Carcinoma, but also described in other carcinoma types)

Invasive carcinoma pattern characterized by cells with tall columnar morphology and reversed polarity, often within stromal spaces

Clinical  Associated with underlying carcinoma type (often Invasive Micropapillary Carcinoma)

Radiology ─ Reflects underlying carcinoma type

Macro  Reflects underlying carcinoma type

Micro ─

 Tumor cells are distinctly tall, columnar, with eosinophilic cytoplasm

 Nuclei often located towards the apical (luminal-facing) aspect of the cell

 Basal aspect of the cell faces the stroma, showing "reversed polarity"

 Cells often form clusters, tubules, or micropapillae lying within clear, retraction-like stromal spaces (similar to Invasive Micropapillary Carcinoma)

 May be admixed with conventional Invasive Micropapillary Carcinoma or other carcinoma types

IHC ─

 Reflects underlying carcinoma type (often (+)ER, (+)PR, frequently (+)HER2 if associated with Invasive Micropapillary Carcinoma)

 (+) EMA shows staining on the stromal-facing (basal) surface, highlighting the reversed polarity (similar to Invasive Micropapillary Carcinoma)

Molecular  Reflects underlying carcinoma type

DDx ─

 Invasive Micropapillary Carcinoma: TCCRP is often considered a variant pattern within this; distinction may be academic if classic IMPC is also present

 Columnar Cell Lesions/FEA: Benign/atypical intraductal lesions; lack invasion; lack reversed polarity EMA staining

 Apocrine Carcinoma: Apocrine cytologic features (granular cytoplasm, prominent nucleoli); typically (+)AR; lacks characteristic reversed polarity pattern

Prognosis  Likely driven by the associated carcinoma type and overall grade/stage; pattern itself associated with aggressive features like lymphovascular invasion and nodal metastasis, similar to Invasive Micropapillary Carcinoma

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Breast  Fibroepithelial & Mesenchymal

Fibroadenoma

Benign biphasic tumor composed of proliferating glandular epithelium and specialized mammary stroma

Clinical  Most common benign breast tumor, typically young women (20s-30s), often presents as palpable, mobile, rubbery mass

Radiology ─

 Mammography: Well-circumscribed, oval or lobulated mass, iso- or slightly hyperdense; coarse "popcorn" calcifications common in involuting fibroadenomas

 Ultrasound: Well-circumscribed, oval, hypoechoic or isoechoic mass, may have gentle lobulations, posterior acoustic enhancement common

Macro  Sharply circumscribed, encapsulated, firm, rubbery, gray-white nodule; cut surface often bulges and may show slit-like spaces

Micro ─

 Biphasic proliferation of benign ductal/glandular epithelium and bland fibrous/myxoid stroma

 Intracanalicular pattern: Stroma compresses ducts into elongated clefts and slits

 Pericanalicular pattern: Stroma surrounds open, rounded ducts/glands

 Often mixed intracanalicular and pericanalicular patterns

 Stroma typically low to moderate cellularity, bland spindle cells, no significant atypia, rare mitoses (<3/10 HPF)

 Epithelium usually simple cuboidal/columnar, may show usual ductal hyperplasia, apocrine metaplasia, cysts; rarely atypical hyperplasia or carcinoma in situ

 Variants: Juvenile (more cellular stroma/epithelium, younger age), Myxoid (abundant myxoid stroma), Complex (cysts >3mm, sclerosing adenosis, epithelial calcifications, or papillary apocrine change)

IHC ─

 Epithelium: (+)CKs (e g, CK7, CK8/18), (+)ER/PR (variable), (-)myoepithelial markers in luminal cells

 Myoepithelium: (+)p63, (+)SMA, (+)calponin, (+)S100 (variable)

 Stroma: (+)CD34, (+)vimentin, (+)ER/PR/AR (variable), (-)CKs

Molecular  MED12 gene mutations common in stromal component

DDx ─

 Phyllodes Tumor (Benign): Increased stromal cellularity (esp subepithelial), leaf-like architecture more prominent, may have >3 mitoses/10 HPF

 Tubular Adenoma: Predominantly closely packed small tubules, minimal stroma

 Hamartoma: Admixture of mature adipose tissue, fibrous stroma, and benign glands/lobules, often less circumscribed

Prognosis  Benign, excellent prognosis; very low risk of malignant transformation (usually carcinoma arising in epithelial component, rarely sarcoma in stroma); complex fibroadenoma associated with slightly increased breast cancer risk

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Phyllodes Tumor

Biphasic fibroepithelial neoplasm characterized by increased stromal cellularity and architectural features resembling intracanalicular fibroadenoma, with potential for recurrence and metastasis (borderline/malignant)

Clinical  Less common than fibroadenoma, typically older women (mean age 40s-50s), usually presents as palpable mass, often larger and may grow more rapidly than fibroadenoma

Radiology ─

 Mammography/Ultrasound: Often large, well-circumscribed, oval or lobulated mass, may have cystic spaces; frequently indistinguishable from fibroadenoma

Macro  Usually well-circumscribed (benign/borderline) or infiltrative (malignant), gray-white, fleshy, firm mass; cut surface shows characteristic leaf-like clefts and protrusions; may have cystic degeneration, hemorrhage, or necrosis, especially in larger/higher-grade tumors

Micro ─

 Biphasic tumor with benign epithelial component lining elongated, compressed ducts and clefts, and a stromal component showing a spectrum of changes

 Characteristic leaf-like architecture (stromal fronds protruding into cystic spaces)

 Stromal cellularity increased compared to fibroadenoma, often with subepithelial condensation (cambium layer)

 Graded based on combination of stromal features (WHO 2012):

 Benign: Mild stromal hypercellularity and atypia, <5 mitoses/10 HPF, pushing margins, no stromal overgrowth

 Borderline: Moderate stromal hypercellularity and atypia, 59 mitoses/10 HPF, pushing or focally infiltrative margins, stromal overgrowth usually absent or focal

 Malignant: Marked stromal hypercellularity and atypia, 10 mitoses/10 HPF, infiltrative margins, stromal overgrowth often present; diagnosis also made if malignant heterologous elements (e g, liposarcoma, chondrosarcoma, osteosarcoma) present

 Stromal overgrowth: Presence of stroma without epithelium in at least one low-power field (x4 objective)

 Epithelial component usually benign, may show hyperplasia, rarely atypia or carcinoma in situ

IHC ─

 Epithelium: (+)CKs

 Stroma: (+)Vimentin, (+)CD34 (often patchy, may be lost in higher grades/stromal overgrowth), variable (+)SMA, (+)desmin, (+)ER/PR/AR; usually (-)CKs, (-)p63 (may be focal); p53 overexpression common in borderline/malignant

Molecular  More complex and numerous genetic alterations than fibroadenoma, especially in borderline/malignant grades; includes gains (e g, 1q) and losses (e g, 9p21, 13q), TP53/RB1 mutations, EGFR/IGF1R amplification; MED12 mutations less frequent than in fibroadenoma

DDx ─

 Fibroadenoma (esp Cellular): Less stromal cellularity/atypia/mitoses, lacks significant stromal overgrowth or infiltrative margins

 Metaplastic Carcinoma (esp Spindle Cell/Sarcomatoid): Malignant spindle cells express CKs and/or p63/p40; lacks typical leaf-like architecture; often triple-negative

 Primary Sarcoma: Purely mesenchymal, lacks epithelial component, very rare in breast

 Periductal Stromal Tumor: Similar stroma but infiltrative growth around open ducts, lacks leaf-like pattern

 Pseudoangiomatous Stromal Hyperplasia (PASH): Anastomosing slit-like spaces in fibrous stroma, lacks leaf-like architecture and stromal atypia/mitoses

Prognosis  Related to grade and margin status; wide excision with negative margins crucial

 Benign: Local recurrence ~1020%, metastasis exceptionally rare

 Borderline: Local recurrence ~1530%, metastasis rare (~<5%)

 Malignant: Local recurrence ~2030%, distant metastasis (hematogenous, typically lung/bone) ~1025%

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Mesenchymal Tumors

Hemangioma

Benign vascular neoplasm composed of capillary or cavernous type vessels

Clinical  Usually incidental finding in older women, rarely palpable mass

Radiology ─ Nonspecific, may appear as well-circumscribed mass or cluster of microcalcifications

Macro  Usually small (<2 cm), soft, red-brown nodule, may be poorly defined

Micro ─

 Well-circumscribed (usually) proliferation of small capillary-sized vessels (capillary hemangioma) or larger dilated vascular channels (cavernous hemangioma)

 Vessels lined by bland, flattened endothelial cells without atypia or significant mitotic activity

 Often located within interlobular stroma, may involve lobules

 Perilobular Hemangioma: Characteristic variant composed of small capillary vessels clustered around lobules

 Thrombosis or phleboliths (calcified thrombi) may be present

IHC ─

 Endothelial cells: (+)CD31, (+)CD34, (+)ERG, (+)Factor VIII-related antigen

 (-)Cytokeratins

 Myoid markers (SMA, Calponin) may highlight pericytes around vessels

DDx ─

 Angiosarcoma (Low-grade): More infiltrative growth, anastomosing vascular channels, mild endothelial atypia, occasional mitoses

 Pseudoangiomatous Stromal Hyperplasia (PASH): Anastomosing slit-like spaces lined by bland spindle cells (myofibroblasts), not true endothelial cells; spaces are (+)CD34 but (-)CD31/ERG in lining cells

 Angiomatosis: Diffuse proliferation of benign vessels involving a large area of breast tissue, poorly circumscribed

Prognosis  Benign, excellent prognosis

Angiomatosis

Diffuse proliferation of benign vascular channels

Clinical  diffuse breast enlargement or ill-defined mass

Radiology ─ Nonspecific

Macro  Poorly defined, spongy area involving parenchyma and adipose tissue

Micro ─

 Diffuse, proliferation of vascular channels permeating breast stroma and adipose tissue

 Vessels vary in size, often capillary or cavernous type, lined by bland endothelial cells

 No significant endothelial atypia or mitotic activity

 Interspersed normal breast ducts and lobules

IHC ─

 Endothelial cells: (+)CD31, (+)CD34, (+)ERG

 (-)Cytokeratins

DDx ─

 Angiosarcoma (Low-grade): Although diffuse, typically shows more infiltrative pattern with anastomosing channels and at least mild endothelial atypia

 Hemangioma: Well-circumscribed lesion, not diffuse

 Vascular Malformation (e g, Arteriovenous Malformation): Contains abnormal thick-walled arteries and veins

Prognosis  Benign, but may recur locally if incompletely excised due to diffuse nature

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Atypical Vascular Lesion (AVL)

Benign endothelial proliferation, typically small, occurring after radiation therapy or chronic lymphedema, distinct from angiosarcoma

Clinical  Usually develops in skin of previously irradiated breast or arm with lymphedema, presents as small red papules or vesicles

Radiology ─ Usually not imaged, skin lesion

Macro  Small (<1 cm) red or violaceous papules, vesicles, or plaques on the skin

Micro ─

 Dermal proliferation of small, simple, thin-walled vascular channels

 May dissect collagen bundles

 Lined by bland endothelial cells, minimal or no atypia, often hobnail appearance

 Mitoses typically absent

 Usually confined to superficial dermis, may extend deeper

IHC ─

 Endothelial cells: (+)CD31, (+)CD34, (+)ERG

 (-)Cytokeratins

 (-)MYC amplification/overexpression (key distinction from post-radiation angiosarcoma)

DDx ─

 Angiosarcoma (Low-grade, especially post-radiation): More infiltrative, anastomosing channels, greater endothelial atypia, possible mitoses, often (+)MYC amplification/overexpression

 Hemangioma: More circumscribed, less likely in post-radiation setting

 Lymphangioma Circumscriptum: Dilated lymphatic channels in dermis

Prognosis  Benign, but requires complete excision due to potential for local recurrence and the critical need to exclude angiosarcoma; some consider it a potential precursor to angiosarcoma

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Angiosarcoma

Malignant neoplasm of endothelial cells forming vascular channels

Clinical  May be primary (younger women, palpable mass, often large) or secondary (older women, post-radiation or chronic lymphedema, often skin lesions)

Radiology ─ Nonspecific, may be ill-defined mass or skin thickening; MRI may show heterogeneous enhancement

Macro  Ill-defined, hemorrhagic, spongy mass or violaceous skin lesions

Micro ─

 Spectrum from low-grade (resembling hemangioma) to high-grade (solid, epithelioid, or spindle cell areas)

 Low-grade: Well-formed, anastomosing vascular channels infiltrating stroma and fat, mild endothelial atypia, scant mitoses

 Intermediate-grade: Increased cellularity, endothelial tufting, papillary formations, occasional mitoses

 High-grade: Solid sheets of atypical endothelial cells, spindle cell areas, prominent papillary formations, necrosis, hemorrhage, frequent mitoses

 Epithelioid variant: Large cells with abundant eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli, mimicking carcinoma

 Post-radiation angiosarcoma often starts in skin, may be multifocal

IHC ─

 Endothelial cells: (+)CD31, (+)CD34, (+)ERG, (+)Factor VIII (variable)

 (+)FLI1

 Epithelioid variant often (+)Cytokeratins (potential pitfall)

 Post-radiation angiosarcoma: (+)MYC amplification/overexpression (highly specific)

 (-)ER, PR, HER2

DDx ─

 Hemangioma/Atypical Vascular Lesion: Less infiltration, lack significant atypia/mitoses; AVL lacks MYC amplification

 Metaplastic Carcinoma (esp Spindle Cell): (+)Cytokeratins, (+)p63; lacks diffuse vascular marker positivity

 Phyllodes Tumor (Malignant): Biphasic, stromal atypia, lacks diffuse vascular marker positivity

 Other Sarcomas: Specific lineage markers (e g, desmin, S100)

Prognosis  Generally poor, high risk of local recurrence and distant metastasis (lung, liver, bone, skin); grade may correlate with prognosis in primary but not secondary angiosarcoma; post-radiation type often aggressive

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Nodular Fasciitis

Benign, reactive proliferation of myofibroblasts, often rapidly growing

Clinical  Uncommon in breast, usually young adults, presents as rapidly growing, sometimes tender, palpable mass

Radiology ─ Nonspecific mass, may be ill-defined

Macro  Usually small (<3 cm), circumscribed or ill-defined, firm, gray-white nodule

Micro ─

 Cellular proliferation of plump, immature-appearing spindle cells (myofibroblasts) arranged in short fascicles or haphazardly

 Characteristic "tissue culture" appearance

 Stroma is typically loose, myxoid, with extravasated red blood cells and scattered lymphocytes

 Mitotic figures often numerous but typically normal morphology

 No significant nuclear atypia

 May show infiltrative borders into adjacent fat or stroma

IHC ─

 Spindle cells: (+)SMA, (+)MSA (variable), (+)Vimentin

 (-)Desmin, (-)S100 protein, (-)Cytokeratins, (-)CD34, (-)Beta-catenin (nuclear)

Molecular  Characteristic USP6 gene rearrangement often present

DDx ─

 Fibromatosis: More infiltrative, less cellular, less myxoid, lacks tissue culture appearance, (+)nuclear Beta-catenin

 Myofibroblastoma: More organized fascicles of bland spindle cells, thick collagen bundles, (+)CD34, (+)Desmin

 Spindle Cell Metaplastic Carcinoma: (+)Cytokeratins, (+)p63/p40; usually older patients

 Low-grade Fibromyxoid Sarcoma/Myxofibrosarcoma: Rare in breast, different morphology and IHC

 Phyllodes Tumor (stromal component): Leaf-like architecture, lacks tissue culture appearance

Prognosis  Benign, self-limited process; may regress spontaneously; local excision is curative, recurrence is rare

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Myofibroblastoma

Benign mesenchymal tumor composed of myofibroblasts

Clinical  Uncommon, typically older men, but also occurs in women (often postmenopausal); presents as painless, well-circumscribed, mobile mass

Radiology ─ Well-circumscribed mass, may mimic fibroadenoma or carcinoma

Macro  Well-circumscribed, firm, rubbery, gray-white nodule, usually <4 cm

Micro ─

 Spindle cells arranged in clusters or short fascicles separated by hyalinized collagen bundles

 Cells have oval to spindle nuclei, inconspicuous nucleoli, scant eosinophilic cytoplasm

 Low mitotic activity, minimal to no atypia

 Variable amount of interspersed mature adipose tissue

 Variants: Cellular, collagenized/fibrous, lipomatous, epithelioid, deciduoid-like, palisaded/Schwannian-like

IHC ─

 Spindle cells: (+)CD34, (+)Desmin, (+)SMA (variable), (+)ER, (+)PR, (+)AR (variable), (+)Bcl2

 (-)Cytokeratins, (-)S100 protein, (-)HMB45

Molecular  Loss of chromosome 13q14 region (includes RB1 gene) characteristic

DDx ─

 Fibromatosis: Infiltrative borders, lacks circumscription, (+)nuclear Beta-catenin, (-)CD34, (-)Desmin

 Spindle Cell Lipoma: Morphologically similar, often more prominent adipose tissue, shares 13q deletion; distinction may be academic

 Solitary Fibrous Tumor: Staghorn vessels, (+)CD34, (+)STAT6, (-)Desmin

 Spindle Cell Metaplastic Carcinoma: (+)Cytokeratins, (+)p63/p40; usually triple-negative

 Nodular Fasciitis: More myxoid, "tissue culture" appearance, lacks thick collagen bundles, (-)CD34, (-)Desmin

Prognosis  Benign, excellent prognosis; local excision is curative, recurrence is very rare

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Fibromatosis (Desmoid Tumor)

Locally aggressive, non-metastasizing fibroblastic/myofibroblastic proliferation

Clinical  Uncommon, wide age range, presents as firm, irregular, fixed mass; may be associated with prior surgery/trauma or Gardner syndrome

Radiology ─ Irregular, spiculated mass, often suspicious for carcinoma

Macro  Ill-defined, very firm, gray-white, fibrous mass with infiltrative borders

Micro ─

 Infiltrative proliferation of bland, slender spindle cells arranged in sweeping fascicles

 Cells have uniform, elongated nuclei, pale cytoplasm, indistinct cell borders

 Collagenous stroma, variable cellularity

 Mitoses rare or absent, no significant atypia

 Characteristic infiltration into adjacent breast parenchyma and adipose tissue, entrapping ducts and lobules

IHC ─

 Spindle cells: (+)Vimentin, (+)SMA (variable), (+)MSA (variable)

 (+)Nuclear Beta-catenin (characteristic, ~80% of cases)

 (+)ER beta (variable), (-)ER alpha, (-)PR

 (-)CD34, (-)Desmin, (-)S100 protein, (-)Cytokeratins

Molecular  CTNNB1 (Beta-catenin) gene mutations common in sporadic cases; APC gene mutations in Gardner syndrome associated cases

DDx ─

 Low-grade Fibromatosis-like Metaplastic Carcinoma: (+)Cytokeratins (may be focal), (+)p63/p40; lacks nuclear Beta-catenin

 Scar Tissue: Less cellular, more hyalinized collagen, history of prior procedure

 Nodular Fasciitis: More cellular, myxoid stroma, "tissue culture" appearance, lacks nuclear Beta-catenin

 Myofibroblastoma: Well-circumscribed, (+)CD34, (+)Desmin, lacks nuclear Beta-catenin

 Phyllodes Tumor (Low-grade): Biphasic, leaf-like architecture, (+)CD34

Prognosis  Benign (does not metastasize), but high rate of local recurrence (2030%) if incompletely excised; requires wide local excision

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Granular Cell Tumor

Benign neural tumor of Schwann cell origin with granular cytoplasm

Clinical  Rare in breast (~6% of all GCTs), wide age range (peak 40s-50s), more common in women of African descent; presents as firm, often fixed mass, may mimic carcinoma clinically and radiologically

Radiology ─ Often irregular or spiculated mass, may mimic carcinoma

Macro  Usually small (<3 cm), firm, ill-defined, gray-white or yellowish nodule

Micro ─

 Infiltrating nests, cords, or sheets of large polygonal cells with abundant, intensely eosinophilic, granular cytoplasm (due to lysosomal accumulation)

 Small, central, round to oval, darkly stained nuclei, minimal atypia

 Indistinct cell borders

 Often infiltrates adjacent stroma and fat, may entrap benign ducts/lobules causing pseudoepitheliomatous hyperplasia of overlying skin or duct epithelium

 Mitoses rare

 Malignant variant exceedingly rare (criteria: necrosis, spindling, vesicular nuclei with large nucleoli, increased mitoses >2/10 HPF, high N/C ratio, pleomorphism)

IHC ─

 Tumor cells: (+)S100 protein (strong, diffuse), (+)CD68 (lysosomal marker), (+)PAS-diastase resistant granules, (+)Inhibin (variable), (+)Calretinin (variable), (+)SOX10

 (-)Cytokeratins, (-)EMA, (-)SMA, (-)Desmin, (-)ER, (-)PR, (-)HER2, (-)GCDFP-15

DDx ─

 Invasive Carcinoma (esp Apocrine or Histiocytoid Lobular): (+)Cytokeratins; Apocrine is (+)AR, (+)GCDFP-15; Histiocytoid Lobular is (-)E-cadherin; both (-)S100

 Fat Necrosis with Histiocytes: Foamy macrophages, lipid vacuoles, associated inflammation; histiocytes (+)CD68 but (-)S100

 Benign Histiocytic Proliferations: Lack S100 positivity

 Alveolar Soft Part Sarcoma (metastatic): Distinct alveolar pattern, PAS+ crystalline material, TFE3+

Prognosis  Benign, excellent prognosis; requires complete excision as infiltrative borders can lead to local recurrence if margins positive; malignant GCT is rare but aggressive

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Pseudoangiomatous Stromal Hyperplasia (PASH)

Benign stromal proliferation characterized by complex, anastomosing slit-like spaces within dense collagenous stroma, mimicking a vascular proliferation

Clinical  Wide age range (premenopausal > postmenopausal), often incidental microscopic finding, may present as palpable mass (tumorous PASH)

Radiology ─ Nonspecific, may be well-circumscribed mass, focal asymmetry, or no abnormality

Macro  Usually incidental finding; tumorous form presents as firm, rubbery, well-circumscribed, gray-white mass

Micro ─

 Proliferation of bland, slender spindle cells (myofibroblasts) within dense, collagenous stroma

 Formation of complex, anastomosing, empty slit-like spaces lined by spindle cells, mimicking vascular channels

 Spaces lack red blood cells

 Spindle cells have scant cytoplasm, uniform nuclei, no atypia or mitoses

 May entrap benign ducts and lobules

 Often associated with fibrocystic changes or fibroadenomas

IHC ─

 Spindle cells lining spaces: (+)CD34, (+)Vimentin, (+)PR (variable), (+)Actin (variable)

 (-)CD31, (-)ERG (confirming spaces are not true vascular channels)

 (-)Cytokeratins, (-)S100 protein

DDx ─

 Low-grade Angiosarcoma: True vascular channels lined by endothelial cells ((+)CD31, (+)ERG), often contain red blood cells, infiltrative growth, endothelial atypia

 Fibroadenoma/Phyllodes Tumor: Biphasic lesions with epithelial component, distinct stromal patterns

 Myofibroblastoma: Discrete tumor composed of fascicles of spindle cells and collagen bundles, (+)Desmin

 Fibromatosis: More cellular fascicles, infiltrative, (+)nuclear Beta-catenin

Prognosis  Benign, no increased risk of malignancy; tumorous PASH may recur locally (~1520%) if incompletely excised, but recurrence is benign

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