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Adenomyoepithelioma
Amyloid tumor
Angiolipoma
Apocrine adenoma of breast
Atypical or benign vascular lesion, post radiation
Benign stromal spindle cell tumor
Benign tumors / changes - Clear cell
Carney syndrome
Cellular fibroadenoma
Chondrolipoma
Cylindroma (dermal type)
Ductal adenoma
Eccrine spiradenoma
Fibromatosis of breast
Galactocele of breast
Gynecomastia-like changes
Hemangiopericytoma of breast
Juvenile xanthogranuloma
Mucocele
Myofibroblastoma of breast
Nodular mucinosis
Sclerosing lobular hyperplasia
Spindle cell lipoma / pleomorphic lipoma
Subareolar sclerosing hyperplasiaAdenomyoepithelioma
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Etiology | Clinical features | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Additional referencesDefinition / general
- Biphasic tumor composed of variable number of myoepithelial cells around small epithelial lined spaces
- First recognized in the breast by Hamperl (Curr Top Pathol 1970;53:161)
Essential features
- Biphasic tumor with epithelial and myoepithelial components
- Benign to low grade malignant behavior and a propensity for recurrence
- Either epithelial or myoepithelial component can show malignant transformation so thorough evaluation recommended
- Wide surgical excision with appropriate margins recommended to prevent recurrence
- Microscopy shows tubular or lobular structures with epithelial and myoepithelial components
- IHC: Epithelial component is cytokeratin+, EMA+, CEA+; myoepithelial component is S100+, SMA+, SMM-HC+, p63+
Terminology
- Other breast myoepithelial lesions are myoepitheliosis and myoepithelioma
- WHO (2012) divides adnomyoepithelioma into a benign type (both components benign) and a form where malignant transformation is seen
- Page recommends that cases with malignant histology not be called adenomyoepithelioma but be defined by the histology of the malignant component (Am J Surg Pathol 2005;29:1294)
Epidemiology
- Uncommon, mean age 60 years
- Rare in male breast
Sites
- Usually occurs in the peripheral portion of the breast, but can be central
- No predilection for either breast
Etiology
- Considered a variant of intraductal papilloma
Clinical features
- Usually presents as a mass
- Usually benign, although may recur locally
- Benign appearing tumors rarely metastasize to lung (Arch Pathol Lab Med 2006;130:1349)
- Malignant tumors are usually low grade; may metastasize to lung, brain, jaws, lymph nodes
- If metastasize, shows hematogenous spread, usually occurs in tumors > 1.6 cm in size (World J Surg Oncol 2013;11:285)
Prognostic factors
- Mitotic rate > 3 MF / 10 HPF is associated with recurrence
- Tubular variant and some lobular variants with high mitotic activity are prone to recurrence
- High mitotic rate, atypia, necrosis, cellular pleomorphism and infiltrative borders favor malignancy
Case reports
- 48 year old woman with collagenous spherulosis in an adenomyoepithelioma of the breast (J Clin Pathol 2004;57:83)
- 56 year old woman with intracystic adenomyoepithelioma of the breast (Breast Cancer 2007;14:429)
- Cellular adenomyoepithelioma of the breast (Am J Surg Pathol 1983;7:863)
- Three patients with breast adenomyoepithelioma (AJR Am J Roentgenol 2003;180:799)
- Diagnostic review of adenomyoepithelioma of the breast (Arch Pathol Lab Med 2013;137:725)
- 50 year old woman with malignant metastatic adenomyoepithelioma of breast (Hematol Oncol Stem Cell Ther 2009;2:364)
- 50 year old woman with malignant adenomyoepithelioma of the breast (Am J Surg Pathol 1998;22:631)
- 60 year old woman with breast adenomyoepithelioma (malignant transformation of epithelial and myoepithelial component, World J Surg Oncol 2013;11:285)
- 68 year old woman with breast mass, present for 14 years, gradually increasing in size (Case of the Week #418)
- 69 year old woman with myoepithelial carcinoma arising in an adenomyoepithelioma of the breast (Pathol Int 2006;56:211)
- 71 year old woman with malignant adenomyoepithelioma of the breast with malignant proliferation of epithelial and myoepithelial elements (Arch Pathol Lab Med 2000;124:632)
- 75 year old woman with rapidly increasing breast mass (Arch Pathol Lab Med 2004;128:235)
- 77 year old woman with malignant adenomyoepithelioma of the breast (Pathol Res Pract 2007;203:599)
- 80 year old woman with malignant adenomyoepithelioma of the breast (Eur J Gynaecol Oncol 2009;30:234)
- Woman with radioresistant malignant myoepithelioma of the breast (J Med Imaging Radiat Oncol 2009;53:234)
Treatment
- Wide local excision with appropriate margins
- May recur with incomplete excision
Clinical images
Images hosted on PathOut server:
Images hosted on other servers:
Mammogram - courtesy of Dr. Mark R. Wick
Images hosted on other servers:
Radiological characteristics of lesion
69 year old woman (fig 1A, 1B)
Gross description
- Well circumscribed, usually small (mean 1 - 2 cm), but can be up to 8 cm
- Firm
- May have satellite nodules
Gross images
Images hosted on PathOut server:
Images hosted on other servers:
Malignant adenomyoepithelioma - COW #418
Unusually large bisected tumor with central
cavity with bosselated contour (AFIP)
Images hosted on other servers:
Intracystic nodule
Microscopic (histologic) description
- Well circumscribed, may be encapsulated or multinodular
- Proliferation of epithelial and myoepithelial cells
- Aggregated lobules of glands with tall lining epithelium with scant eosinophilic cytoplasm and hyperchromatic nuclei surrounded by myoepithelial cells with clear cytoplasm
- Epithelial cells usually form glandular spaces
- Myoepithelial cells can be dominant and may be spindle shaped, clear or polygonal
- Apocrine metaplasia may be present, also adenomyoepitheliomatous hyperplasia
- Variants include spindle cell type (epithelial lined spaces may be sparse, resembles leiomyoma), tubular variant (ill defined margins, may resemble tubular adenoma), lobulated variant (nests of myoepithelial cells surround compressed epithelial lined spaces)
- Malignant appearing cases have local invasion, high mitotic rate, severe atypia (Am J Surg Pathol 1992;16:868)
Microscopic (histologic) images
Scroll to see all images.
Images hosted on PathOut server:
AFIP Images:
Images hosted on other servers:
Benign histology but malignant behavior:
Malignant histology:
Malignant adenomyoepithelioma - COW #418
CK7 - COW #418
34βE12 - COW #418
CD117 - COW #418
p63 - COW #418
Smooth muscle myosin heavy chain - COW #418
Courtesy of Dr. Mark R. Wick
AFIP Images:
Intraductal bands of pale myoepithelium separate adenomatous ductal epithelium
Myoepithelial cells with clear cytoplasm crowd glandular epithelium and expand into stroma
Overgrowth of myoepithelium in two nodules separated by stromal band
Myoepithelial cells in small clusters have replaced most ductal elements
Foci of glands with luminal secretions
Isolated myoepithelial cells with clear vacuolated cytoplasm
Transition between adenomatous and myomatous growth
AE1+ glandular cells, (myoepothelial cells are negative)
Images hosted on other servers:
Proliferation of epithelial and myoepithelial cells
Multilobulated outlines and pushing margins
CK7 (A); p63 (B); CK5 (C); S-100 (D)
69 year old woman (fig 1C)
72 year old woman (fig 2C)
74 year old woman (fig 3C, 3D, 3E)
Solid sheets and cords of round, oval cells
Various images and stains
With collagenous spherulosis
Benign histology but malignant behavior:
Benign appearing tumors that metastasized to lung
Lung metastases with benign histology
Malignant histology:
Mitotic activity
Various images
Keratin+ epithelium
AE1-AE3, S100
Cytology description
- Moderate to highly cellular with large clusters of epithelium and myoepithelium
- Tubular structures occasionally found
- Myoepithelium appears as small clusters or dispersed cells with epithelioid morphology, intranuclear or intracytoplasmic vacuoles, often naked bipolar nuclei
- Mild to moderate nuclear atypia present
- Metachromatic fibrillary stroma occasionally found
- No mitotic figures, no necrosis
- Often classified incorrectly as fibroadenoma, suspicious for malignancy or malignant (Cancer 2006;108:250)
Cytology images
Images hosted on other servers:
Various images
Positive stains
Negative stains
Electron microscopy description
- Myoepithelial features (classic) include myofibrils with dense bodies, pinocytotic vesicles, desmosomes or tight junctions, patchy basement membrane
Molecular / cytogenetics description
- Case report with t(8;16)(p23;q21) (Cancer Genet Cytogenet 2005;156:14)
Differential diagnosis
- Adenosis tumor: no prominent myoepithelial component
- Intraductal papilloma: no prominent myoepithelial component
- Invasive carcinoma (on core biopsy): unequivocal evidence of invasion
- Nipple adenoma: no prominent myoepithelial component
- Tubular adenoma: very well circumscribed (tubular variant is not), myoepithelial cells are inconspicuous or rare
Additional references
Amyloid tumor
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Pathophysiology | Etiology | Diagrams / tables | Clinical features | Laboratory | Radiology description | Radiology images | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosis | Additional referencesDefinition / general
- Extracellular mass-like deposition of amorphous and insoluble proteins in an abnormal fibrillary configuration
Essential features
- Extracellular deposition of acellular eosinophilic material in fat, stoma or blood vessels creating a mass effect
- Stains positively for Congo red; metachromatic on Wright’s stain
- May be localized or part of systemic amyloidosis
- Treatment by complete excision
Terminology
- Amyloidosis in the breast first reported by Fernandez and Hernandez in 1973 (J Clin Pathol 2002;55:634)
- Also called amyloidoma
Epidemiology
- Very rare
- Usually women ages 45 - 79 years
Sites
- 80% occur in right breast
- Bilateral or unilateral, but only rarely is confined to breast
Pathophysiology
- Abnormal deposition of insoluble protein in an abnormal fibrillary configuration
- Most common types are amyloid light chain (AL) and amyloid A (AA)
- AL is caused by immunoglobin light chains and is secondary to plasma cell dyscrasia
- AA is reactive amyloidosis and is secondary to chronic inflammatory disease
- In vast majority of patients, breast amyloidosis is part of a systemic AL type disease (usually kappa light chain proteins)
- It can be associated with malignancies of the breast
Etiology
- Depends on the type of amyloidosis:
- AL type amyloidosis – plasma cell dyscrasia (myeloma, Waldenstrom’s macroglobulinemia)
- AA type amyloidosis – chronic inflammatory disease (rheumatoid arthritis, Reiter’s syndrome)
Diagrams / tables
Images hosted on other servers:
Figure 7: Flow chart
for diagnosis of amyloidosis
Clinical features
- Painless mass
- May occur late in systemic disease
- Complete physical examination needed to differentiate primary and secondary amyloid tumors
Laboratory
- See diagram above
Radiology description
- Visualized as mass on ultrasound and MRI
Radiology images
Images hosted on other servers:
Figures 1 and 2
Case reports
- 58 year old woman with amyloid tumor mimicking breast carcinoma (South Med J 2008;101:199)
- 59 year old woman with sclerosing lymphocytic lobulitis and amyloid of the breast (Breast 2006;15:281)
- 60 year old woman with amyloid tumor in silicone implanted breast (Plast Aesthet Res 2016;3:240)
- 76 year old woman with amyloid deposition of breast (AJR Am J Roentgenol 2000;175:1590)
- 77 year old woman with bilateral breast masses (Arch Pathol Lab Med 2004;128:e67)
- Amyloid deposition of the breast in primary Sjögren syndrome (JBR-BTR 2006;89:313)
Treatment
- Excisional biopsy is successful if amyloid tumor is limited to the breast
Gross description
- 5 cm or less, firm, gray white, opalescent
Microscopic (histologic) description
- Amorphous eosinophilic extracellular deposits in fat, stroma or vessels
- May cause ductal atrophy, form rings around individual fat cells, have associated multinucleated giant cell reaction or osseous metaplasia (J Clin Path 2002;55:634)
Microscopic (histologic) images
Images hosted on PathOut servers:
Courtesy of Mark R. Wick, M.D.
Amyloid occurs
in lobule (lower left)
and as separate
stromal nodule
(upper right, AFIP)
Thick wavy bands
of amyloid around
lobular glands and
a nodular deposit that
is virtually acellular
Thioflavin T stain (left) and parenchyma - congo red (right), courtesy of Mark R. Wick, M.D.
Images hosted on other servers:
Various images
Fig 2: H&E, Fig 3: Congo Red
Amyloid with osseous metaplasia
Cytology description
- Amorphous acellular material with scattered plasma cells, lymphocytes, stromal cells and epithelial cells
- Rare multinucleated giant cells
- Metachromatic by modified Wright’s stain (Diagn Cytopathol 2003;28:325)
Cytology images
Images hosted on other servers:
Dense hyaline material and giant cells
Positive stains
- Congo red (red orange with apple green birefringence under polarized light)
- Metachromatic with crystal violet
Negative stains
Electron microscopy description
- Straight, nonbranching, haphazard amyloid fibrils, 5 - 10 nm, mixed with collagen fibers (Am J Surg Pathol 1986;10:539)
Differential diagnosis
- Plasmacytoma: immature plasma cells, no amyloid
- Stromal fibrosis: may have hyaline material, but it has different histologic features from amyloid; also Congo red negative
Additional references
Angiolipoma
Table of Contents
Definition / general | Terminology | Sites | Etiology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stainsDefinition / general
Terminology
Sites
Etiology
Clinical features
Radiology images
Prognostic factors
Case reports
Treatment
Clinical images
Various images
Microscopic (histologic) description
Microscopic (histologic) images
Anastomosing capillaries, inconspicuous endothelial cells (AFIP)
Soft tissue (AFIP): mature adipose tissue and prominent vasculature
Capillary vessels with microthrombi
Courtesy of Dr. Mark R. Wick
Breast angiolipoma
Cytology description
Apocrine adenoma of breast
Table of Contents
Definition / general | Essential features | Terminology | Etiology | Clinical features | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosis | Additional referencesDefinition / general
- Adenoma with apocrine cytology throughout
- Rare adenoma that is:
- Composed exclusively of benign apocrine cells (homogeneous)
- Sharply demarcated from surrounding breast tissue
- Contains only epithelial proliferative elements
- Has minimal supportive stroma
Essential features
- Nodular mass composed exclusively of apocrine cells without cytological atypia
- Sharply demarcated from adjacent breast tissue and has minimal stromal component if any
- Considered benign, excision is curative
- Cytological atypia, necrosis and invasive features suggest atypical hyperplasia/apocrine malignancy (DCIS / invasive), recommend careful evaluation of the entire lesion
Terminology
- Pure breast adenomas with apocrine differentiation were first described by Hertel et al in 1976 (Cancer 1976;37:2891)
Etiology
- May represent nodular sclerosing adenosis with apocrine differentiation
Clinical features
- Can present as a mass
Prognostic factors
- Generally accepted to be benign, however, the number of cases reported is not sufficient to determine the level of risk associated (J Clin Pathol 2007;60:1313)
Case reports
- 45 year old man with 3 mm tumor (Arch Pathol Lab Med 2003;1 527:1498)
- 47 year old woman with mammographically detected tumor (WV Med J 2008;104:16)
- 53 year old woman with coexisting invasive ductal carcinoma (Pathol Res Pract 2007;203:809)
Treatment
- Complete excision is curative
Microscopic (histologic) description
- By definition, composed exclusively of benign apocrine cells (homogeneous), sharply demarcated from surrounding breast tissue, containing only epithelial proliferative elements, with minimal supportive stroma
- Localized nodular focus of tubular, papillary and cystic apocrine metaplasia; benign glands have abundant granular eosinophilic cytoplasm, apical luminal blebs and decapitation secretion
- May contain calcifications (Pathology 2001;33:149)
Microscopic (histologic) images
Images hosted on Other servers:
Apocrine adenoma
Apocrine adenoma (6 mm tumor)
Apocrine adenoma (well circumscribed)
Tubular apocrine adenoma, skin
Cytology description
- Can be cuboidal or flattened, two distinct types seen:
- Cytoplasm granular and strongly eosinophilic, supranuclear vacuole containing yellow brown pigment (rich in iron / hemosiderin)
- Globoid and pale nuclei with 1-2 prominent nucleoli (nuclei may become hyperchromatic in flattened epithelium as in tension apocrine cysts)
- Cytoplasm distinctly foamy with small vacuoles that may coalesce and show lipofuscin pigment in cytoplasm
- Central nuclei with 1-2 prominent nucleoli
- Cytoplasm granular and strongly eosinophilic, supranuclear vacuole containing yellow brown pigment (rich in iron / hemosiderin)
- Recommend caution to exclude apocrine DCIS or invasive apocrine carcinoma if necrosis / atypia / mitosis present
Positive stains
Differential diagnosis
- Apocrine DCIS: extensive proliferation, marked nuclear pleomorphism, multiple prominent nucleoli and comedo-type necrosis
- Atypical apocrine hyperplasia: architectural atypia such as Roman bridges, cribriform patterns and multiple papillary fronds; no connective cores or cytological atypia such as three fold variation in nuclear size and marked pleomorphism
- Fibroadenoma: prominent proliferating stromal component, compressed epithelium in intracanalicular variant
- Prominent apocrine changes as a part of fibrocystic changes: not nodular, no distinct mass
- Well differentiated apocrine carcinoma: has obvious malignant changes
Additional references
Atypical or benign vascular lesion, post radiation
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Etiology | Clinical features | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosisDefinition / general
- Vascular proliferations that develop after external radiotherapy for breast carcinoma, within field of radiation (Am J Clin Path 1994;102:757)
Essential features
- Benign atypical vascular proliferations developing after external radiation for breast carcinoma
- Typically involves skin overlying breast
- Two types: 1) Lymphatic type; 2) Vascular type
- Differentiate from angiosarcoma by well circumscribed nature, lack of multi layering, mitosis and hemorrhage and absence of extension into subcutaneous tissue
- Vascular type is higher risk for subsequent development of angiosarcoma
- Complete excision and follow up for recurrence is standard treatment
Terminology
- “Atypical vascular lesion†term coined by Fineberg and Rosen in 1994 (Am J Clin Path 1994;102:757)
- Note: atypical vascular pattern refers to a dermoscopic pattern that helps differentiate benign cutaneous lesions from melanoma
Epidemiology
- Mean age 61 years
- Develops 1 - 12 years (mean 6 years) after therapy in radiation field
Sites
- Breast exposed to previous radiation, usually in the skin area
Etiology
- May have lymphatic origin (Histopathology 1999;35:319)
Clinical features
- May recur within radiation field (Cancer 2007;109:1584)
Prognostic factors
- May progress to angiosarcoma, particularly vascular type (Am J Surg Pathol 2008;32:943, Am J Surg Pathol 2005;29:983)
Case reports
- Two women, ages 42 and 50 year old, with post radiation atypical vascular lesions of breast (Case Reports in Pathology 2012:2012;710318)
- 49 year old woman with post radiation atypical vascular proliferation (J Clin Aesthet Dermatol 2011;4:47)
Treatment
- Complete excision and careful follow up for recurrence
Clinical images
Images hosted on other servers:
Left breast is erythematous with sclerotic tissue changes
Figs 1 and 4: Papules and nodules in and around the mastectomy scar
Gross description
- One or more circumscribed papules, bluish purple nodules, small vesicles or erythematous plaques, usually in irradiated field, median 0.5 cm
- Frequently multiple synchronous lesions with discoloration
Microscopic (histologic) description
- Relatively well circumscribed, anastomosing growth pattern of irregular slit-like vascular spaces dissecting dermal collagen but not extending into subcutis
- Lined by single layer of endothelial cells without atypia
- Often micropapillary tufts
- Resembles benign lymphangioendothelioma or patch stage Kaposi sarcoma (Am J Surg Pathol 2002;26:328)
- Lymphatic type:
- Predominantly thin walled, variably anastomosing lymphatics primarily in superficial dermis
- Vascular type:
- Predominantly small, irregularly dispersed, capillary type vessels, invested by pericytes, often blood filled, in superficial or deep dermis
- Associated with extravasated red blood cells or hemosiderin, minor lymphatic type component (Arch Pathol Lab Med 2009;133:1804)
- Higher risk for angiosarcoma (Am J Dermatopathol 2005;27:301, Am J Surg Pathol 2008;32:943)
- Features of angiosarcoma which are lacking in AVLs:
- Multi layering of endothelial cells, prominent nucleoli, mitoses, hemorrhage, destruction of adnexa and extension into subcutaneous tissues
Microscopic (histologic) images
Images hosted on other servers:
Figures 9 - 11
H&E, CD31 and FLI1
Lesions confined to papillary and reticular dermis
Dermis showed perivascular lymphocytic infiltrate and collagenization
Negative stains
Molecular / cytogenetics description
- No MYC amplification (Mod Pathol 2012;25:75)
Differential diagnosis
- Angiosarcoma, well differentiated:
- Atypical endothelial cells, infiltrative margins, usually high grade, larger (median 7.5 cm vs. 0.5 cm for atypical vascular lesions), positive nuclear staining for c-MYC
- Atypical vascular lesions are well circumscribed, not infiltrative, no multi layering, no mitosis, no hemorrhage
- However, angiosarcoma may be part of morphologic continuum with atypical vascular lesions (J Am Acad Dermatol 2007;57:126), and may need additional biopsies to distinguish these entities
- Hobnail hemangioma: smaller, more superficial and more localized
- Lymphangioendothelioma: has intravascular papillary stromal projections that resemble papillary endothelial hyperplasia
Benign stromal spindle cell tumor
Table of Contents
Definition / general | Terminology | Pathophysiology | Etiology | Clinical features | Case reports | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Differential diagnosisDefinition / general
Terminology
Pathophysiology
- Fibroblastic
- Myofibroblastic
- Fibrohistiocytic
- Mixed forms (Pathol Res Pract 2001;197:453)
Etiology
- V: vimentin
- VA: vimentin and actin
- VAD: vimentin, actin and desmin
- VADM: vimentin, actin, desmin and myosin (Am J Surg Pathol 1998;22:141)
Clinical features
Case reports
Gross description
Microscopic (histologic) description
- Spindle cells, hyalinized collagen bundles and adipocytes
- Can have cellular pleomorphism
- Based on the ratio of components and pleomorphism, subclassified as classic, collagenized, epithelioid, cellular, lipomatous and variants resembling solitary fibrous tumor
- Closely packed, spindle shaped cells with indistinct cell margins arranged around thin-walled irregular branching vessels
- The latter may have a 'staghorn' configuration
Microscopic (histologic) images
Spindle cell carcinoma, CD10, S100, SMA
Myofibroblastoma, CD34, S100
Spindle cell lipoma, CD34
Leiomyosarcoma, desmin
Myoepithelial carcinoma, S100, CD10
Cytology description
Positive stains
Differential diagnosis
- Desmin negative, positive for Actin and focal CD34
Benign tumors / changes - Clear cell
Table of Contents
Definition / general | Essential features | Etiology | Clinical features | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesDefinition / general
- Very rare tumor in breast of epithelioid to spindle cells with clear to eosinophilic granular cytoplasm and distinct cell borders, positive for melanocytic markers
Essential features
- Rare tumor differentiating towards Perivascular Epithelioid Cells
- Belongs to PEComa family of tumors, extremely rare in breast
- Microscopy shows spindle to epithelioid cells with clear or eosinophilic cytoplasm and distinct cell borders
- Stains positive for melanocytic markers
- Treatment by excision; new drug rapamycin under investigation
Etiology
- Differentiates towards Perivascular Epithelioid Cell
- Other PEComas are angiomyolipoma, lymphangiomyoma, lymphangioleiomyomatosis, renal capsuloma and clear cell myomelanocytic tumor of the falciform ligament / ligamentum teres
Clinical features
- Benign behavior
Case reports
- 16 year old girl (Am J Surg Pathol 2002;26:670)
Treatment
- Excision
- Trial with rapamycin in progress; may become useful for malignant cases
Microscopic (histologic) description
- Epithelioid to spindle cells with clear cytoplasm and distinct cell borders
Microscopic (histologic) images
Images hosted on other servers:
Clear cell "sugar" tumor - lung
Lung tumor is HMB45+
Positive stains
Molecular / cytogenetics description
Differential diagnosis
- Glycogen rich (clear cell) carcinoma: large nests of clear cells with hyperchromatic nuclei, mild / moderate atypia and pleomorphism; invasive; keratin+, PAS+ diastase sensitive; negative for HMB45 and MelanA
Additional references
Carney syndrome
Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Radiology description | Radiology images | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Additional referencesDefinition / general
- Autosomal dominant syndrome of myxomas / myxoid lesions, spotty skin pigmentation and endocrine overactivity (Am J Surg Pathol 1991;15:713)
- Also considered a familial multiple neoplasia and lentiginosis syndrome
- Linked to PRKAR1A gene (Atlas of Genetics and Cytogenetics) at 17q23-24 (found in 46% of Carney syndrome cases) and CNC2 gene at 2p16 (J Med Genet 2003;40:268)
- First described in 1985 by J. Aidan Carney (Medicine (Baltimore) 1985;64:270, WhoNamedIt.com)
Terminology
- Also called Carney complex
Epidemiology
- Incidence of 70 cases per 100,000 individuals
- Usually white, no gender preference
- Mean age at diagnosis is 10 - 20 years (eMedicine > Cardiology > Carney Complex)
Clinical features
- Myxomas / myxoid fibroadenomas, spotty skin pigmentation and endocrine overactivity
- Also intraductal breast adenoma, spotty pigmentation present from birth, endocrine overactivity, psammomatous melanotic schwannoma (Am J Surg Pathol 1991;15:722)
- Ductal adenoma: palpable painless mass near the areola
- Myxomas occur in heart, but can also occur on the skin (eyelid, external ear canal and nipple) and breast
- Myxomas occur as nodular masses
- ~40% of the patients have multiple and bilateral breast myxomas (breast myxomatosis)
- Bone lesions, osteochondromyxomas or “Carney bone tumor†may also occur
Radiology description
- Myxoma: on mammography appears as well defined, noncalcified, isodense or hypodense mass
- Ultrasound may show well circumscribed, ovoid mass to complex cystic lesion
- Ductal adenoma: soft tissue tumor with coarse microcalcifications, which may be irregular in a linear orientation
Radiology images
Images hosted on other servers:
Hyperintense
nodules corres-
ponding to myxoid
fibroadenomas
Two low density
masses in female
patient with
Carney complex
Solid, ovoid, well circumscribed, hypoechoic lesion in breast
nodules corres-
ponding to myxoid
fibroadenomas
masses in female
patient with
Carney complex
Clinical images
Images hosted on other servers:
Pedunculated, flesh
colored cutaneous
myxoma on trunk
Microscopic (histologic) description
- Myxoma: in reticular dermis, bland stellate and short spindle shaped cells in myxoid (mucinous) stroma with prominent blood vessels
- Ductal adenoma: tubules (longitudinal and parallel) in fibrous stroma
- The tubules are lined by a single row of uniform epithelial cells with vesicular nuclei and eosinophilic cytoplasm
- Secretions may be present in the tubular lumen
Microscopic (histologic) images
Images hosted on other servers:
Breast myxoma at 5× magnification
200×; H&E stain
Polypoid neoplasm of
fibrillary collagen and uniform
stellate cells within abundant
connective tissue mucin
Additional references
Cellular fibroadenoma
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Clinical features | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Differential diagnosis | Additional references | Board review question #1 | Board review answer #1Definition / general
- Resembles fibroadenoma with pericanalicular pattern but more cellular
Essential features
- Fibroadenoma (stromal and epithelial process) with uniform epithelial and stromal hypercellularity but lacking leaf-like pattern of phyllodes tumor
- No atypical features, i.e. no periductal stromal condensation, no cytologic atypia, no mitotic figures > 3/10 HPF
- Excision is curative
Terminology
- Also called juvenile fibroadenoma
- Giant fibroadenoma: terminology used inconsistently; refers to size, often children, but not a distinct histopathologic entity
Epidemiology
- Usually black females, median age 15 years (range 10 - 39 years)
Clinical features
- Grows rapidly but benign behavior
- Often massive size
- Either solitary or multiple / successive lesions; recurrences usually cease in 20's
- Not associated with subsequent carcinoma
Case reports
- 12 year old premenarche girl with bilateral, rapidly enlarging breast lumps (Pathology Research International 2011;2011:1)
- 15 year old girl with bilateral tumors (National Institute of Oncology and Radiobiology, Cuba)
- 18 year old post pubertal girl presented with bilateral, rapidly enlarging breast lumps (Sch J App Med Sci 2016;4:1830)
Treatment
- Conservative excision (preserve as much normal breast tissue as possible, Saudi Med J 2007;28:137, Breast J 2000;6:418)
Gross description
- Yellowish tan, softer than classic fibroadenoma
Gross images
Images hosted on other servers:
Well circumscribed
lobulated mass
Microscopic (histologic) description
- Resembles fibroadenoma with pericanalicular pattern, may be mixed with intracanalicular pattern
- Uniformly hypercellular stroma, no atypical features; i.e. no periductal increase in cellularity, no stromal overgrowth, no cytologic atypia, no mitotic figures > 3/10 HPF
- Frequently epithelial as well as myoepithelial hyperplasia
- Stromal and epithelial balance is not altered; lacks leaf-like growth pattern
- Epithelium may have tufted pattern or hyperplasia characteristic of gynecomastia; may have epithelial atypia (Am J Surg Pathol 1987;11:184)
- No increase in periductal cellularity that is seen in phyllodes tumors
Microscopic (histologic) images
Images hosted on other servers:
Juvenile fibroadenoma
Juvenile fibroadenoma - stromal and epithelial hyperplasia
Mitosis - infrequent finding
Fibroepithelial tumor with florid intraductal epithelial hyperplasia
(Myo)fibroblastic stroma, duct with columnar epithelium
Positive calponin
p63
Cytology images
Images hosted on other servers:
Monolayered sheets of ductal cells
Monolayered sheets of epithelial cells
Differential diagnosis
- Hamartoma: more abundant adipose tissue; epithelial component is more disorganized
- Phyllodes tumor: prominent stromal overgrowth, intracanalicular growth pattern, periductal concentration of cells, variable atypia and mitotic figures
Additional references
Board review question #1
Which feature supports a diagnosis of phyllodes tumor over cellular fibroadenoma?
A. Pericanalicular pattern
B. Young patient
C. Periductal condensation of stromal cells
D. Epithelial tufting or hyperplasia
A. Pericanalicular pattern
B. Young patient
C. Periductal condensation of stromal cells
D. Epithelial tufting or hyperplasia
Board review answer #1
C. Features supporting a diagnosis of phyllodes tumor over cellular fibroadenoma are an older patient, intracanalicular pattern with leaf like formations, periductal condensation of stromal cells, cytologic atypia and increased mitotic activity
Chondrolipoma
Table of Contents
Definition / general | Terminology | Epidemiology | Pathophysiology | Clinical features | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Molecular / cytogenetics description | Molecular / cytogenetics images | Differential diagnosisDefinition / general
Terminology
Epidemiology
Pathophysiology
Clinical features
Case reports
Treatment
Gross description
Gross images
Hand mass
Shoulder mass
Microscopic (histologic) description
Microscopic (histologic) images
Hand mass:
Soft tissue (not breast) - AFIP:
Well circumscribed tumor composed of clusters of small to medium cells with vacuolated cytoplasm; mature fat cells are present
Cytology description
Molecular / cytogenetics description
Molecular / cytogenetics images
t(11;16)(q13;p12-13), 3-way arrangement
with chromosomes 1, 2 and 5
Differential diagnosis
Cylindroma (dermal type)
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Pathophysiology | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosis | Additional referencesDefinition / general
- Very rare tumor of breast that resembles dermal counterpart (Am J Clin Path 2005;123:866)
- Breast is considered a modified sweat gland, hence it can give rise to sweat gland-type neoplasms
Essential features
- Benign skin adnexal tumor, rarely can arise in breast
- Histologically identical to dermal counterpart, with nests and trabeculae of cells in a "jigsaw" puzzle pattern
- Nests show peripheral myoepithelial cells (p63+), central basaloid cells (CK7+), duct-like lumina with or without secretions and are peripherally lined by thick basement membrane material (PAS-D+ and Collagen IV+)
- Important to differentiate from adenoid cystic carcinoma
Terminology
- Also called dermal analog tumor
- "Cylindroma" was first used by Billroth in 1959 (Int J Trichology 2013;5:83), based on nests of cells surrounded by hyaline, resemble a cylinder in cross section
- Cylindroma of the breast was first described in 2001 by Gokaslan (Am J Surg Pathol 2001;25:823)
Epidemiology
- May be associated with turban tumor syndrome (Brooke-Spiegler syndrome, OMIM - Brooke-Spiegler syndrome)
- Brooke-Spiegler syndrome is characterized by multiple skin appendage tumors such as cylindromas, trichoepitheliomas and spiradenomas; the gene responsible for multiple cylindromas, CYLD, is localized to band 16q12-q13
Pathophysiology
- Exact cellular origin unknown; most likely a very primitive sweat gland tumor differentiating towards apocrine line
Case reports
- 61 year old woman with cylindroma of the breast (Int J Trichology 2013;5:83)
- 62 year old woman with cylindroma of the breast (Diagn Pathol 2009;4:30)
- 63 year old woman with solitary cylindroma (dermal analog tumor) of the breast (Am J Surg Pathol 2001;25:823)
Treatment
- Excision
Clinical images
Images hosted on PathOut server:
Contributed by Dr. Mark R. Wick
Images hosted on other servers:
Screening mammogram
Ultrasound screening
Gross description
- Small, well demarcated
- Often near nipple and lactiferous ducts (Am J Surg Pathol 2004;28:1070)
Microscopic (histologic) description
- Jigsaw pattern of epithelial basaloid islands with focal squamous and myoepithelial differentiation
- Islands are bordered by thickened basement membrane, contain hyaline globules
- Also reactive dendritic Langerhans cells that permeate islands, clusters of sebaceous cells and a few eccrine ducts
- No nuclear pleomorphism, no mitotic figures
Microscopic (histologic) images
Images hosted on PathOut server:
Contributed by Dr. Mark R. Wick
Images hosted on other servers:
Basaloid cells in jigsaw puzzle pattern
Jigsaw appearance with nests of basaloid cells surrounded by dense hyaline basement membrane
CK7 highlights central larger cells
p63 highlights peripheral cells
Dermal cylindroma
Skin
PAS, skin
Cytology description
- Small uniform cells with finely granular cytoplasm and oval nuclei
- Some cells are arranged around cylinders of dense acellular material, resembles adenoid cystic carcinoma (Acta Cytol 2004;48:853)
Positive stains
Differential diagnosis
- Adenoid cystic carcinoma, solid variant: atypia, mitotic figures, invasive growth; no continuous thickened basement membrane (Am J Clin Path 2005;123:866)
- Basal cell carcinoma: almost always attached to epidermis, peripheral palisading, no cylinders of dense acellular material
Additional references
Ductal adenoma
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Etiology | Clinical features | Radiology description | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosis | Additional referencesDefinition / general
- Sclerotic intraductal papilloma with pronounced adenomatous featuers (Breast Cancer 2006;13:354)
- First described in 1984 (J Pathol 1984;144:15)
Essential features
- Well circumscribed benign intraductal proliferation of tubules (no papillary component)
- May show calcification, hemorrhage, infarction, squamous or apocrine metaplasia or myxoid change
- May show pseudoinfiltrative pattern (exclude invasion by showing intact myoepithelial marker staining, eg. p63, SMMA)
- Excision is curative
Terminology
- Also called sclerosing papilloma
Epidemiology
- Uncommon
- Usually age 60+ years
- Ductal adenomas with tubular features may occur as part of Carney complex (Am J Surg Pathol 1991;15:722, Am J Surg Pathol 1996;20:1154)
Etiology
- May evolve by sclerosis of intraductal papillary lesion (Hum Pathol 1989;20:903)
Clinical features
- Presents as palpable lump or mammographic abnormality
- May resemble carcinoma at imaging, frozen or permanent section (Breast Cancer 2006;13:354)
Radiology description
- Mammography shows well to ill defined mass with/without calcification
Case reports
- 32 and 64 year old women with ductal adenoma of the breast (Breast Cancer 2006;13:354)
- 66 year old woman with ductal adenoma of the breast with chondromyxoid change (Pathol Int 2002;52:239)
Treatment
- Excision is curative
Gross description
- Single or multiple rounded lesions
- Gritty texture and elastic streaks mimic carcinoma
Microscopic (histologic) description
- Well circumscribed, bounded by dense fibrous wall of medium to large duct from which it arises
- Composed of proliferating epithelial tubules but no papillary component (or it would be a papilloma)
- Has both epithelial and myoepithelial cell types
- May have pseudoinfiltrative pattern due to compressed tubules, which resembles invasion in a core biopsy
- Also focal dilated tubules, epithelial hyperplasia, cysts, squamous or apocrine metaplasia, myxoid change, calcification
Microscopic (histologic) images
Images hosted on PathOut server:
Central fibrosis, peripheral bland ducts
Nodular lesion, primarily spindle cells
Spindle cells with residual ducts
Spindled myoepithelial cells around peripheral duct
Ducts lined by apocrine cells
Images hosted on Other servers:
Ductal adenoma, partially sclerosed
Cytology description
- May resemble lactating adenoma, intraductal papilloma or carcinoma
- Highly cellular with monolayered sheets of ductal cells with cytoplasmic vacuoles that are small and punched out
- Numerous large fragments of purple stroma are adjacent to epithelial cells, forming finger-like hyaline structures or globules between cells
- Nuclei are round / oval with bland chromatin
- Occasional cells have enlarged nuclei with prominent nucleoli
- Also naked oval nuclei (Diagn Cytopathol 1994;10:143, Diagn Cytopathol 1995;13:252)
Positive stains
- Tubular luminal cells: keratin
- Tubular basal cells: S100, actin, p63
- Basement membrane: laminin, type IV collagen
- Stromal spindle cells (myofibroblasts): actin and vimentin (Pathol Res Pract 1993;189:515)
Differential diagnosis
- Intraductal papilloma: papillary, not hyperplastic
Additional references
Eccrine spiradenoma
Table of Contents
Definition / general | Essential features | Epidemiology | Sites | Etiology | Clinical features | Radiology description | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Additional referencesDefinition / general
- Rare, benign, adnexal tumor of skin first reported in 1956 (AMA Arch Derm 1956;73:199)
- Resembles dermal counterpart, with lobules containing biphasic cell population (packed, monotonous, basaloid epithelial cells with scant cytoplasm and round/oval nuclei and peripheral smaller cells with hyperchromatic nuclei)
Essential features
- Benign adnexal tumor
- Circumscribed lesion with lobulated pattern composed of biphasic cell population
- Immunostains p63+, calponin (outer cells) and CK7, CD117 (inner cells)
- Wide local excision is curative
Epidemiology
- Most patients in their 2nd to 4th decade
Sites
- Usually trunk or extremities, rarely other sites
Etiology
- Arises from cutaneous sweat glands
Clinical features
- Palpable, occasionally painful lump
- Benign, rarely undergoes malignant transformation (Arch Pathol Lab Med 1996;120:501)
Radiology description
- Although mammographic and ultrasonographic findings are similar between eccrine spiradenoma of breast and epidermal inclusion cyst, MRI findings differ and can be used to differentiate
Case reports
- 39 year old woman with eccrine spiradenoma arising from breast skin (Case Rep Pathol 2015;2015:615158)
- 43 year old woman with recurring breast tumor with features of eccrine spiradenoma (Am J Clin Pathol 1996;106:665)
- 47 year old woman with eccrine spiradenoma misdiagnosed as an epidermal inclusion cyst (Korean J Radiol 2011;12:256)
- 48 year old woman with malignant eccrine breast spiradenoma (Int J Surg Case Rep 2015;15:81)
- 57 year old woman with spiradenocarcinoma of breast arising in a long standing spiradenoma (Ann Diagn Pathol 2004;8:162)
- Malignant eccrine spiradenoma metastatic to intramammary lymph node (Breast Cancer 2008;15:175)
Treatment
- Wide local excision
Clinical images
Images hosted on Other servers:
Well defined, isodense nodule
Hypoechoic mass
Microscopic (histologic) description
- At low power, appears as a solid neoplasm composed of a single mass or a few masses of basaliod cells
- At higher magnification, two distinct populations of neoplastic epithelial cells can be seen making a circumscribed lesion with a lobulated pattern
- Jigsaw puzzle shaped lobules contain packed, monotonous, larger basaloid epithelial cells with scant cytoplasm and round/oval and pale nuclei
- At periphery are smaller cells with dark hyperchromatic nuclei
- No epidermal connection is seen
Microscopic (histologic) images
Images hosted on Other servers:
Two cell population
Deep dermis
Site unknown
Malignant transformation
Various images
p63, CK7, CD117
Ki67
Cytology description
- Bland groups of uniform cuboidal cells with scant cytoplasm, round/oval nuclei, indistinct nucleoli
- Occasional rosette-like structures (Diagn Cytopathol 1992;8:366)
Additional references
Fibromatosis of breast
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Clinical features | Radiology description | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Board review question #1 | Board review answer #1Definition / general
- Rare benign entity with clonal proliferation of fibroblasts and myofibroblasts similar to counterparts elsewhere
Essential features
- Rare benign entity characterized by spindle cell proliferation of fibroblasts and myofibroblasts as well as varying amounts of collagen
- No metastatic potential, however, it can have local recurrences
- Radiologically and grossly appears as a mass lesion
- Histology shows low grade myofibroblastic proliferation
- Treatment by wide local excision
Terminology
- Also referred to as desmoid tumor or low grade fibrosarcoma
Epidemiology
- Rare (Usually women of childbearing age, may be associated with trauma (Hum Pathol 2009;40:1564)
- Rarely reported in men (see Case reports below )
- Associated with Gardner syndrome, familial multicentric fibromatosis syndrome, silicone and saline breast implants and incidental and surgical trauma (Breast J 2006;12:66, AJR Am J Roentgenol 2005;185:488)
Sites
- May arise in mammary gland or in chest wall musculoaponeurotic tissue and extend into breast
Clinical features
- Firm, painless, mobile mass with or without skin / nipple retraction
- Infiltrative, locally recurrent if positive margins (25%) but non metastasizing (Am J Surg Pathol 1979;3:501, Am J Surg Pathol 1987;11:38)
Radiology description
- Irregularly shaped, high density with spiculated margin on mammography
- Ultrasound shows a poorly defined hypoechoic mass with posterior acoustic shadowing and an echogenic rim, closely mimicking cancer (AJR Am J Roentgenol 2005;185:488)
Case reports
- 22 year old woman with desmoid tumor of the breast (J Plast Reconstr Aesthet Surg 2010;63:339)
- 30 year old woman with breast lump (Proc (Bayl Univ Med Cent) 2013;26:22)
- 39 year old woman with recurrent fibromatosis of the breast (World J Surg Oncol 2006;4:32)
- 39 year old woman with aggressive fibromatosis of the breast (Arch Pathol Lab Med 1985;109:260)
- 70 year old woman with fibromatosis (desmoid tumor) of the breast mimicking a case of ipsilateral metachronous breast cancer (World J Surg Oncol 2006;4:57)
- Fibromatosis of the breast (Breast J 2006;12:66)
Treatment
- Wide local excision with careful attention to negative margins, although may recur even with negative margins (Ann Surg Oncol 2008;15:274)
Clinical images
Images hosted on Pathout server:
Images hosted on other servers:
Mammogram, courtesy of
Dr. Mark R. Wick
Images hosted on other servers:
Associated distortion and tenting of the underlying pectoralis muscle
Gross description
- Ill defined, white-tan-gray fibrous tissue
Gross images
Images hosted on other servers:
Solid ill defined mass
Underlying chest wall
musculature, ribs and
parietal pleura
Microscopic (histologic) description
- Irregular, nonencapsulated proliferation of spindle cells forming interlacing fascicles with variable collagen deposition and cellularity
- Usually extends into surrounding fat and glandular parenchyma
- May have focal lymphoid aggregates at periphery
- May contain eosinophilic inclusions similar to those of infantile digital fibromatosis
- No / rare mitotic figures
- No epithelial component
Microscopic (histologic) images
Scroll to see all images
Images hosted on Pathout server:
Images hosted on other servers:
Images hosted on Pathout server:
Courtesy of Dr. Mark R. Wick
Neoplastic infiltrate surrounds ducts and lobules
Sparsely cellular collagenous area with lymphocytes
Spindle cells with uniform nuclei and no mitotic activity
Keloidal collagen surrounds atrophic duct
Spindle cells in myxoid stroma
Focally, cells have large hyperchromatic nuclei
Images hosted on other servers:
Various images
Bland spindle cells and collagen
Mammary ductal epithelium
Myofibroblastic spindle cells
Low grade myofibroblastic proliferation
Invading muscle
Bland fibroblasts of moderate cellularity
Fig A: infiltration into adjacent breast tissue
Fig B: infiltration into skeletal muscle
Fig C: monotonous, bland spindle cells with abundant
extracellular collagen, occasional small nucleoli
Cytology description
- Hypocellular spindle cells with no / rare glandular elements
Positive stains
- Vimentin, smooth muscle actin (focal)
Negative stains
Electron microscopy description
- Fibroblasts and myofibroblasts
Molecular / cytogenetics description
- Nuclear accumulation of beta-catenin in stromal tumor cells (82%), somatic alterations of APC / beta-catenin pathway (79%, Hum Pathol 2002;33:39)
Differential diagnosis
- Benign fibrous histiocytoma (superficial): storiform pattern of spindled and bland fibroblasts and histiocyte-like cells in mid dermis and subcutaneous tissue with infiltrative margins but sparing epidermis
- Benign stromal spindle cell tumors: circumscribed, no collagen, CD34+
- Inflammatory myofibroblastic tumor: prominent inflammatory infiltrate
- Metaplastic carcinoma - fibromatosis-like variant: epithelial component is atypical, keratin+
- Nodular fasciitis: superficial, well circumscribed, zonation effect with hypocellular central region and hypercellular periphery; feathery, tissue culture like growth pattern due to abundant ground substance; often with mucoid pools (microcysts)
- Phyllodes tumor: stromal component
Board review question #1
- A 39 year old woman presents with a 3 cm left breast mass with skin retraction. Histological examination of the needle core biopsy shows a predominantly benign spindle cell proliferation intermixed with varying amounts of collagen. A rare focus shows benign glandular elements. SMA is positive; ER, PR is negative. What would be your recommendation for this patient?
- Benign lesion, no further treatment needed
- Phyllodes tumor, needs lumpectomy
- Tumor reduction by chemoradiation prior to surgery
- Wide local excision
Board review answer #1
- D. Wide local excision - fibromatosis should be treated by wide local excision to prevent local recurrences. No other therapy is needed unless the lesion is unresectable due to its extension into surrounding structures.
Galactocele of breast
Table of Contents
Definition / general | Essential features | Etiology | Radiology description | Radiology images | Case reports | Treatment | Microscopic (histologic) description | Cytology imagesDefinition / general
- Milk filled retention cyst beneath areola, common in young women who are pregnant or breast feeding
- Due to abrupt cessation of lactation
Essential features
- Milk filled retention cyst due to blockade of breast duct
- Occurs in young women who are pregnant or breast feeding
- Mass lesion on imaging, mimics lipoma or cancer (history is helpful)
- FNA / histology shows fat/protein contents, inflammation, debris and secretory epithelial cells
- Benign; diagnostic aspiration may be curative
Etiology
- Blockage of breast duct due to lactation
Radiology description
- Mammogram: oval circumscribed mass with high radiolucency due to high fat content (mimics lipoma)
Radiology images
Nodule
Case reports
- Male infant with hyperprolactinemia and bilateral galactocele (Int J Pediatr Endocrinol 2009;2009:578610)
- 36 year old woman with mass arising in accessory breast tissue in axilla (Arch Gynecol Obstet 2007;276:379)
- 37 year old nulliparous woman with prolactinoma (Maturitas 2009;62:98)
- 54 year old postmenopausal woman (Breast Cancer Res 2000;2:A53)
- Due to augmentation mammoplasty (Aesthetic Plast Surg 2005;29:274)
Treatment
- Aspiration or no treatment
Microscopic (histologic) description
- Dilated, anastomosing, epithelium lined channels, often with secretory activity
- Cyst contents may leak into adjacent tissue and cause lipogranulomatous reaction with foamy macrophages (J Cutan Pathol 2010;37:973)
Cytology images
27 year old woman with 1.5 cm nontender breast nodule and FNAC
Gynecomastia-like changes
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Clinical features | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosis | Additional referencesDefinition / general
- Increased ducts and prominent stroma, histologically identical to gynecomastia of the male breast
Essential features
- Young patients with palpable mass lesion, typically negative mammography
- Microscopy shows poorly circumscribed lesion with ductal hyperplasia and micropapillae, periductal fibrosis, periductal edema (similar to male gynecomastia)
- No lobules seen
- Often adjacent fibrocystic changes present
- Benign, no increased risk for malignancy
Terminology
- Also called gynecomastoid hyperplasia
Epidemiology
- 0.15% of female breast lesions (Arch Pathol Lab Med 2001;125:506, Arch Pathol Lab Med 2000;124:844)
Clinical features
- Mean age 32 years, patients present with palpable mass associated with fibrocystic changes in adjacent breast
Clinical images
Images hosted on Other servers:
Bilateral mammograms
1-cm noncalcified nodule
Retroareolar mass
Retroareolar tissue
Enlarging asymmetric density
Microscopic (histologic) description
- Poorly circumscribed areas of ductal hyperplasia with micropapillae, periductal stromal fibrosis or edema and slight lymphocytic infiltrate
- Involves one low power field or entire core fragment of at least 1 cm, without terminal duct lobular units present (same as male gynecomastia)
- No associated mammary hamartomatous changes, no areas of juvenile hyperplasia, no juvenile fibroadenoma
Microscopic (histologic) images
Images hosted on Other servers:
Proliferation of ducts with periductal fibrosis but without lobules
Mild lymphocytic infiltrate
Ducts but no lobules
Epithelial proliferations
Myoepithelial layer
Edematous periductal stroma
Differential diagnosis
- Hamartoma: sharply circumscribed, has lobules, variable smooth muscle or cartilage, no periductal stromal changes
- Pubertal macromastia: young patients, massive involvement
Additional references
Hemangiopericytoma of breast
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Pathophysiology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Additional referencesDefinition / general
- Rare, low grade malignant tumor with circumscribed area of bland spindled cells with branching and staghorn vessels
- Originally regarded as a vascular, pericyte derived tumor; recently categorized by WHO as a fibroblastic tumor similar to solitary fibrous tumor
Essential features
- Low grade malignant tumor composed of branching / staghorn vessels and bland spindled cells
- Complete excision with negative margins is considered curative
- Aggressive behavior suggested if large (> 5 cm), necrosis, mitosis, infiltrative margins
Terminology
- First described by Stout and Murray in 1942 (Ann Surg 1942;116:26)
Epidemiology
- Very rare
- Usually women 33 - 67 years
Pathophysiology
- Originates from capillary pericytes
Clinical features
- Painless, firm nodule / mass, not attached to the skin
Radiology images
Images hosted on Other servers:
Well circumscribed mass
Hypoechoic lesion
Prognostic factors
- Nuclear atypia, high cellularity or necrosis, increased mitotic index (>4/10 HPF), large tumor size (>5 cm) and infiltrative margins are considered criteria for malignancy, and also correlate with local recurrence and metastatic disease
Case reports
- 33 year old woman with tumor of pectoralis major muscle mimicking a breast mass (Int J Surg Case Rep 2013;4:338)
- 43 year old woman (Case Rep Oncol Med 2015;2015:210643)
- 66 year old woman (Arch Gynecol Obstet 2008;277:357)
- Metastasized hemangiopericytoma of the breast (Arch Gynecol Obstet 2009;280:491)
Treatment
- Complete local excision with negative margins
- Inadequate data regarding lymph node dissection; in general, lymph node metastases are rare
- Mastectomy may be necessary for large lesions (Mod Pathol 1988;1:98)
Microscopic (histologic) description
- Thin walled, staghorn-type vessels separated by polygonal cells with indistinct margins, pale cytoplasm and small round/oval nuclei
- May have areas of hemorrhage and cystic degeneration
- Variable mild increase in mitosis
Microscopic (histologic) images
Images hosted on PathOut server:
Thin walled staghorn-type vessels
Reticulin
Images hosted on Other servers:
Fusiform tumor cells
CD34 and vimentin
Pectoralis major mass
Sinonasal tumor
Cytology description
- Cellular
- Single and tightly packed clusters of oval to spindled cells aggregating around branched capillaries
Negative stains
Electron microscopy description
- Pericytes associated with vessels (Am J Surg Pathol 1981;5:745)
Additional references
Juvenile xanthogranuloma
Table of Contents
Definition / general | Terminology | Epidemiology | Radiology images | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosis | Additional referencesDefinition / general
- Benign histiocytic disorder of skin, very rare in breast tissue itself
- See also topic under Soft Tissue Tumors
Terminology
- Also called nevoxanthoendothelioma
Epidemiology
- Benign histiocytic disorder of infants and children; 15% occur in adults (usually young)
Radiology images
Image hosted on other servers:
Figure 2. Breast: Mammogram (A)
Sonogram (B)
Doppler sonography (C)
Case reports
- 20 year old woman with clinical extranumerary nipple (Case of the week #5)
- 60 year old woman with bilateral xanthogranulomas (J Ultrasound Med 2007; 26:535)
- 74 year old woman with prior cutaneous juvenile xanthogranulomas and breast masses (Am J Surg Pathol 2005;29:827)
- Occurring in woman within field of radiation therapy for breast cancer (J Cutan Pathol 2010;37:891)
Treatment
- Conservative excision
Gross description
- Cutaneous lesions usually 0.1 to 2.0 cm, yellow-red and papulonodular
Microscopic (histologic) description
- Dense dermal infiltrate of lymphocytes, histiocytes, Touton giant cells (usually), eosinophils and neutrophils, which may extend into subcutis; late - epidermis thins out and the rete ridges become elongated
- Extracutaneous lesions may lack the Touton giant cells
- Note: histiocytic giant cells may be Touton type [ring of nuclei surrounding foamy cytoplasm with cytoplasm usually also visible around the nuclei; Langhans type [nuclei form a horseshoe arrangement, not necessary a distinct category from Touton type] or foreign body type [haphazard nuclear arrangement]
- May have calcifications and variable amounts of fibrosis
Microscopic (histologic) images
Images hosted on PathOut server:
Images hosted on other servers:
Images hosted on other servers:
Scalp
Figure 3: Diffuse nodular aggregation of histiocytes
intermingled with chronic inflammatory cells
Differential diagnosis
- Benign fibrous histiocytoma: related entity, usually has a storiform pattern
- Inflammatory pseudotumor: may have ganglion like cells, usually positive for SMA, Vimentin, Factor XIIIa (>90%) keratin and ALK (50-60%)
- Spindle cell metaplastic carcinoma: HMW keratin positive in about half cases
- Xanthoma: uniform collection of foam cells and variable Touton giant cells, no other inflammatory cells, often associated with hyperlipidemia
Additional references
Mucocele
Table of Contents
Definition / general | Essential features | Epidemiology | Clinical features | Radiology description | Prognosis and treatment | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Differential diagnosis | Additional referencesDefinition / general
- Rare lesion characterized by dilated epithelium lined ducts filled with mucin, often associated with extravasation of mucin into the stroma
- Cysts lined by flat or low cuboidal epithelium
- Pathogenesis is unknown, but excessive mucinous secretions or ductal obstruction may be responsible
Essential features
- Characterized by extravasated acellular mucin in periductal stroma
- Although there is variable associated hyperplasia of the cyst lining in mucocele-like lesion, there are no epithelial cells "floating" within the luminal or extravasated mucin, which is a is critical finding in distinguishing mucocele-like lesion from mucinous carcinoma of the breast
- Although originally described as a benign lesion, associations with ADH, DCIS and invasive carcinoma have been reported in several studies
- This finding emphasizes the importance of searching for atypia when mucocele-like lesion is present
- Pure mucocele-like lesion without atypia typically has benign behavior
Epidemiology
- Mean age 40 years, range 25-61 years (Am J Surg Pathol 1986;10:464)
Clinical features
- Many reports have described an association between mucocele-like lesion and the simultaneous presence of ADH, DCIS or mucinous carcinoma (Human Pathology 2016;49:33, Mod Pathol 2011;24:683, Diagn Pathol 2011;6:29, Histopathology 2013;62:894, Arch Patol Lab Med;115;137, Am J Clin Pathol 2012;138:783, N Eng J Med 2005;353:229)
- Mucin extravasation or mucocele-like lesions at core biopsy warrant radiological-pathological correlation and close follow-up is recommended to rule out any atypical lesion (Histopathology 2009;55:609)
Radiology description
- There is no definitive test or imaging for differentiating mucocele-like lesion from other suspicious lesions
- Most lesions are initially identified as indeterminate calcifications on mammogram (AJR 2006;186:1356)
- Sonographic finding of mucocele-like lesion most commonly reported is a cystic mass (AJR 2011;196:1424)
Prognosis and treatment
- Mucocele-like lesion on core biopsy warrants close radiological-pathological correlation
- Pure mucocele-like lesion without atypia is usually associated with a benign outcome (Histopathology 2013;62:894)
- Several studies have recommended close clinical follow-up as an alternative to surgical excision in women with a core biopsy of mucocele-like lesion without atypia and no associated mass (Am J Clin Path 2012;138:783, Ultrasonography 2015;34:133)
- Risk of associated malignancy is much higher if atypia is present
- Compete surgical excision recommended in certain situations where core needle biopsy reveals the presence of associated ADH/DCIS or where a mass with indistinct and irregular margins is shown by mammography or sonography (Clinical Imaging 2011;35:94, Diagnostic Pathol 2011;6:29)
Gross images
Mucin filled cysts contained DCIS
Microscopic (histologic) description
- Microscopic examination of the entire specimen is important to rule out any atypia / malignancy
- Mucin containing cysts that often rupture, with extravasation of mucin into surrounding stroma
- Epithelium lining the cysts may be benign/flat, hyperplasia, ADH, DCIS or mucinous carcinoma
- Myoepithelial cells adhere to strips of cells floating in lakes of mucin
- Calcifications are often present
Microscopic (histologic) images
Images hosted on other servers:
Mucocele-like lesions
Mucocele-like tumor
With focal columnar cell hyperplasia
With ADH
Cytology description
- Poorly cellular with cohesive clusters of bland cells in two dimensional sheets with abundant mucoid background, no / rare intact single tumor cells, no atypia if mucocele only (Am J Surg Pathol 1999;23:552, Am J Clin Pathol 1991; 95:875)
- Excisional biopsy is necessary to confirm diagnosis (Breast Cancer 2009;16:77)
- The most important features favoring benign MLL over mucinous carcinoma on FNA are: (1) younger patient, (2) cells arranged in cohesive monolayers, (3) no significant nuclear atypia, (4) scant cellularity, (5) no or rare single intact tumor cells
- Mucinous carcinomas are usually more cellular with more single tumor cells, 3D clusters, mild/moderate nuclear atypia, and a solid mass by imaging (Cytopathology 2004;15:104, Acta Cytologica 2000;44:765)
Differential diagnosis
- Mucinous carcinoma: prominent luminal cell proliferation and variable number of tumor cells “floating†within the mucin; in mucocele-like lesions, luminal mucin is devoid of epithelial cells
- Cystic mastopathy: associated with prominent apocrine differentiation
- Florid duct ectasia with luminal mucin: generally contains lipid rich material within ducts with prominent foamy histiocytes, in contrast to acellular mucin seen in mucocele-like lesion
Additional references
Myofibroblastoma of breast
Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Clinical features | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Molecular / cytogenetics description | Electron microscopy description | Differential diagnosisDefinition / general
- Rare benign spindle cell tumor of mammary stroma composed of myofibroblasts
- First described in 1987 (Am J Surg Pathol 1987;11:493)
Terminology
- Also called benign stromal spindle cell tumor with predominant myofibroblastic differentiation
Epidemiology
- Although initially considered male predominant, now equal gender frequency (older men and post-menopausal women, Mod Pathol 1996;9:786)
- Incidence appears to be increasing, but probably an artifact of mammographic screening (Arch Pathol Lab Med 2008;132:1813)
Etiology
- May derive from CD34+ vimentin+ fibroblasts of mammary stroma capable of multidirectional differentiation (Histopathology 2003;42:233)
- Probably related to spindle cell lipoma and solitary fibrous tumor (Virchows Arch 2002;440:249, Am J Surg Pathol 2001;25:1022, Breast J 2008;14:287)
Clinical features
- Solitary, slow growing nodule
- Benign behavior
Case reports
- 10 month old boy (Fetal Pediatr Pathol 2012;31:164)
- 25 year old man with bilateral gynecomastia (Int J Surg Pathol 2001;9:331)
- 40 year old woman (Indian J Pathol Microbiol 2008;51:395)
- 57 year old woman with 2.4 cm breast mass (Case of the Week #395)
- 59 year old woman (University of Pittsburgh, Case #249)
- 65 year old man with 15 cm rapidly growing tumor (J Med Case Rep 2008;2:157)
- 72 year old man (Arch Pathol Lab Med 2003;127:e415)
- 73 year old man with cellular tumor (Am J Mens Health 2012;6:344)
- 83 year old man with 10 cm tumor (Am J Surg Pathol 1994;18:1170)
- Post-radiation tumor (Breast J 1999;5:136)
Treatment
- Excision is curative
Clinical images
Images hosted on other servers:
15 cm tumor
Gross description
- Well-circumscribed nodule or multilobar mass
- Bulging, gray-pink and 1-4 cm
- Resembles fibroadenoma
Gross images
Images hosted on other servers:
Firm, circumscribed, tan-gray lobulated nodule
Microscopic (histologic) description
- Well-circumscribed
- Uniform, bland spindle cells haphazardly arranged in fascicles with pushing borders, separated by broad bands of hyalinized collagen (Stanford University)
- Spindle cells have abundant eosinophilic cytoplasm, round / oval nucleus with 1-2 small nucleoli
- May have mild nuclear pleomorphism
- Prominent mast cells
- Variants include cellular, collagenized, epithelioid (Am J Surg Pathol 2009;33:1085), fatty (J Clin Pathol 2001;54:568) and infiltrative (no atypia or mitotic activity)
- May have histiocytoid cells, prominent vessels, focal cartilaginous differentiation (Virchows Arch 1999;434:547), smooth muscle and multinucleated floret-like giant cells
- No / rare mitotic activity
- Usually no entrapment of ducts or lobules
- Usually no necrosis
Microscopic (histologic) images
Scroll to see all images.
Images hosted on PathOut server:
Case of the Week #395:
H&E images
CD34
ER
PR
Well circumscribed
Fascicles of spindle cells separated by dense collagen
Images hosted on other servers:
Epithelioid type
Well circumscribed
Spindle cells and hyalinized collagen
Classic type
Cellular variant
Other variants
Predominantly fatty variant
CD34+
H&E, CD34, SMA
Fig 1: sharply circumscribed tumor with fibrous pseudocapsule
Fig 2: bland spindle cells in collagenous or myxoid stroma
Fig 3A: CD34+; fig 3B: bcl2+
Fig 4: desmin+ (focal)
Cytology description
- May appear malignant
- Cohesive and single spindle cells without atypia
- Scant cytoplasm and oval nuclei with fine granular chromatin, occasional nuclear grooves and small nucleoli (Diagn Cytopathol 2004;30:406)
- Tumor cells may be intimately associated with extracellular matrix material
- Also mast cells, hyaline bands between tumor cells (Diagn Cytopathol 2002;26:290)
- May have features of solitary fibrous tumor or pleomorphic lipoma (Pathol Res Pract 1999;195:257)
Cytology images
Images hosted on other servers:
Fig 2: loose groups of cells with abundant
eosinophilic granular cytoplasm and bland nuclei
Positive stains
- ER, PR (Histopathology 2000;36:515), vimentin and CD34 (often)
- bcl2
- Variable desmin, caldesmon and androgen receptors (Hum Pathol 1998;29:347)
Negative stains
- S100, cytokeratin
- Ki-67(or low)
Molecular / cytogenetics description
- 13q-, 16q- (similar to spindle cell lipoma, J Pathol 2000;191:282)
Electron microscopy description
- Features of smooth muscle and myofibroblasts: rough endoplasmic reticulum, bundles of myofilaments with focal densities, intermediate filaments and attachment plaques (Ultrastruct Pathol 1999;23:249, Pathol Res Pract 1999;195:1)
Differential diagnosis
- Fibromatosis: not circumscribed, more diffuse fibrosis and no thick bands of collagen
- Nodular fasciitis: more infiltrative, mucoid stroma
- Low grade myofibroblastic sarcoma: marked cellular pleomorphism, infiltrating margins and high mitotic rate
- Myoepithelioma: S100+, keratin+
Nodular mucinosis
Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Radiology images | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosis | Additional referencesDefinition / general
Terminology
Epidemiology
Clinical features
Radiology images
Case reports
Treatment
Gross description
Microscopic (histologic) description
Microscopic (histologic) images
Various images
Various images
Cytology description
Positive stains
Negative stains
Differential diagnosis
- May be accompanied by atypical ductal hyperplasia or intraductal carcinoma
Additional references
Sclerosing lobular hyperplasia
Table of Contents
Definition / general | Epidemiology | Clinical features | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Differential diagnosisDefinition / general
- Benign proliferative tumor of young women, characterized by enlarged lobules with an increased number of acini and variable interlobular fibrosis
Epidemiology
- Incidence is 3%
- Most often young African-American women (Arch Pathol Lab Med 2005;129:1345)
- There are no evident risk factors
Clinical features
- Presents as localized, palpable, firm, well circumscribed tumor up to 5 cm in diameter, usually in upper outer quadrant (Cytojournal 2006;3:8, Bocker: Preneoplasia of the Breast, 1st Edition, 2006, Chapter 2)
- Causes local pain
Radiology description
- Mammography: non-specific findings of well defined mass or normal mammogram
- May be obscured by relatively denser glandular tissues in younger patients
- Sonography: usually hypoechoic mass (Eur Radiol 2003;13:1645)
- Microcalcifications may be seen occasionally, especially in older women
Prognostic factors
- Benign; not associated with an increased risk for carcinoma (Arch Pathol Lab Med 2005;129:1345)
Case reports
- 12 and 16 year old girls (Cytojournal 2006;3:8, Diagn Cytopathol 2004;31:340)
- 20 year old woman with well circumscribed lump in left breast (Arch Pathol Lab Med 2005;129:1345)
- 45 year old woman with painless 4 cm tumor of left breast (Malays J Pathol 2011;33:129)
- 9 patients (Eur Radiol 2003;13:1645)
Treatment
- Surgical excision is curative
- There are no follow up studies documenting the frequency of recurrence, but it has been suggested that these lesions may recur as a fibroadenoma (Hoda: Rosen's Breast Pathology, 4th Edition)
- Natural regression is suspected because no lesions have been found in postmenopausal women (AJR Am J Roentgenol 1995;165:291)
Gross description
- Relatively well circumscribed, soft to firm, nodular mass
- Cut surface is solid, gray-white with a few scattered slit-like spaces
- Usually lacks the sharp demarcation and whiteness of a fibroadenoma
- No hemorrhage or necrosis
Gross images
Images hosted on PathOut server:
White, firm and homogeneous
Microscopic (histologic) description
- Well circumscribed but not encapsulated
- Enlarged breast lobules with an increased number of acini
- The acini have the normal breast architecture with distinct single layers of epithelial and myoepithelial cells
- Intralobular stroma is collagenized, with variable interlobular stromal fibrosis
- Sclerosing lobular hyperplasia is found in surrounding breast tissue of 50% of fibroadenomas (Hum Pathol 1984;15:336)
- No / rare mitotic figures, no atypia
Microscopic (histologic) images
Images hosted on PathOut server:
Well circumscribed
Large lobules, increased ductules, dense stroma with collagenous fibrosis, bland and acellular
Prominent fibroblastic and myofibroblastic proliferation forming slit-like spaces, resembles pseudoangiomatous stromal hyperplasia
Dense interlobular fibrosis has glomeruloid pattern with trichrome stain
CK8+ lobules
Myoepithelial cells are calponin+
Images hosted on Other servers:
Well circumscribed
Large lobules
Dense keloid-like
Normal lobular architecture
Cytology description
- Cytology by itself is not diagnostic
- Uniform round/oval ductal epithelial cells in monolayered sheets and round acinar clusters; no atypia (Diagn Cytopathol 2004;31:340, Cytojournal 2006;3:8)
- Occasional stromal fragments and a fair number of bare nuclei
- No branched tubular fragments
- Complete absence of stromal fragments and rarity of bare nuclei are not consistent features of sclerosing lobular hyperplasia, and may not help to distinguish from fibroadenoma
Cytology images
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FNA, moderately cellular
Scattered fragments of stroma
Round to oval nuclei
Differential diagnosis
- Fibroadenoma: more clearly demarcated; irregular proliferating elongated and distorted ducts and loose cellular stroma; both are treated similarly
- Hamartoma: clearly demarcated, lacks the enlarged lobular architecture of sclerosing lobular hyperplasia; may contain fatty and myoid component
- Tubular adenoma: tight packing of acinar structures (no or few ductal elements), with little intervening stroma
Spindle cell lipoma / pleomorphic lipoma
Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Radiology description | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Differential diagnosisDefinition / general
- A rare variant of breast lipoma characterized by a mixture of mature adipocytes, collagen forming spindle cells and varying degrees of myxoid change
- The first report citing breast as a possible location of benign spindle cell tumor was by Toker et al in 1981 (Cancer 1981;48:1615)
Terminology
- Also termed benign stromal spindle cell tumor with predominant adipocytic component (Tumors of the Mammary Gland (AFIP Atlas of Tumor Pathology: Series 4)
Epidemiology
- Usually age > 40 years; no gender preference
- Although pleomorphic lipoma was initially considered a separate entity, it is now accepted that both tumors are part of a spectrum of neoplasms characterized by similar clinical, morphologic and genetic features (J Cancer Res Clin Oncol 1994;120:707)
Etiology
- Recent studies have shown that cellular angiofibroma, spindle cell lipoma and mammary-type myofibroblastoma harbor a monoallelic deletion of Rb1 and FOXO1, and share similar morpho-immunophenotypical features, suggesting a genetic link (Mod Pathol 2011;24:82, Histopathology 2007;51:410, Virchows Arch 2006;449:244, Cancer Genet Cytogenet 2007;177:131, Virchows Arch 2002;440:249)
Radiology description
- Usually appears on mammogram as a well circumscribed mass
- On ultrasound, usually a homogeneously hyperechoic solid mass or nodule
Case reports
- 28 year old woman (Pathology 1999;31:288)
- 48 year old woman (Indian J Pathol Microbiol 2008;51:234)
- Aspiration biopsy of pleomorphic lipoma of the breast (Acta Cytol 2000;44:255)
Treatment
- Benign tumors are managed by routine follow up
- Malignant or potentially malignant tumors should be completely excised
Gross description
- Usually 3 to 5 cm, but can be up to 14 cm
- Most tumors present as a single, well demarcated, firm, unencapsulated mass
- Cut surface is pale, white to gray with a variably lipomatous and myxoid component
- Calcification, hemorrhage and necrosis are not present
Gross images
White fibrous tumor
Microscopic (histologic) description
- Spindle cell lipoma typically contains three components: thin, uniform spindle cells arranged in short, parallel bundles, mature adipocytes and ropy birefringent collagen
- Myxoid background appears as a thin, amorphous and semitransparent substance
- Very low rate of mitosis, no necrosis
- Pleomorphic lipomas have prominent floret cells
Microscopic (histologic) images
Images hosted on PathOut server:
Courtesy of Dr. Mark R. Wick
Images hosted on Other servers:
Spindle cells associated with collagen
Cytology, H&E, CD34
Spindle cells are CD34+
Spindle cells are desmin-
Spindle cells are SMA-
Cytology description
- Benign spindle cell tumor: presence of spindle cells without ductal epithelial cells
- Pleomorphic lipoma: Clusters of multinucleated floret-like giant cells with hyperchromatic nuclei; also round cells and few spindle shaped cells (Acta Cytol 2000;44:255)
Negative stains
Differential diagnosis
- Cellular angiofibroma:
- Usually described in genital region
- Striking haphazard and hyalinized vessels in a wispy collagenous, occasionally myxoid stroma are distinctive for cellular angiofibroma (Mod Pathol 2011;24:82)
- Low grade myofibroblastic sarcoma:
- Fascicles of ill-defined spindle cells with infiltrative margins (J Clin Oncol 2005;23:6249)
- Pleomorphic nuclei and numerous mitotic figures are common
- Immunohistochemically, at least one myogenic marker (desmin, SMA, Calponin) is positive
- Metaplastic spindle cell carcinoma:
- Sheets of spindle shaped cells or sarcomatoid elements (Jpn J Clin Oncol 1997;27:46)
- Keratin+ and p63+
- Myofibroblastoma:
- Although breast spindle cell lipomas are typically immunoreactive to CD34 and vimentin, they are not reactive to desmin and smooth muscle actin as seen in myofibroblastoma (Virchows Arch 2002;440:249)
Subareolar sclerosing hyperplasia
Table of Contents
Definition / general | Clinical features | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisDefinition / general
- A sclerosing papillary variant of radial sclerosing lesion in the subareolar region, first described in 1987 (Cancer 1987;59:1927)
- Beneath areola, without involvement of surface of nipple (called nipple adenoma if nipple is involved)
- Note: this is the only article describing this lesion in the literature
Clinical features
- 26 to 73 years, mean 46 years
- A mass located beneath the nipple or areola in the breast
- Left and right breast affected equally
- Erosion or ulceration of the nipple are absent
- Nipple retraction may occur
- Several patients had blood discharge
- Nonspecific mammographic findings, may mimic carcinoma
Treatment
- Excision through circumareolar incision, sparing the nipple
- Recurrence may occur after incomplete excision
- Benign, no evidence that this condition is premalignant
Gross description
- Firm to hard, round to oval tumor with indistinct margins
- Mean 1.2 cm, range 0.6 to 2.0 cm
- Yellow streaks may be noted
Microscopic (histologic) description
- Prominent central elastosis and sclerosis in the center of the tumor, duct hyperplasia is more prominent in the periphery, causing distortion of the ductal pattern
- Cartilaginous metaplasia may occur in the sclerotic core
- Varying amounts of papillary ductal proliferation
- Papillary epithelial hyperplasia within ducts may exhibit considerable atypia
- Generally no cystic change, no papilary apocrine change, no squamous metaplasia
Microscopic (histologic) images
Images hosted on PathOut server:
Papillary duct proliferation
Solid foci in ducts
Papillary duct hyperplasia
Differential diagnosis
- Nipple adenoma / florid papillomatosis of the nipple: typically more nodular than satellite; apocrine metaplasia, squamous cyst, squamous metaplasia may occur
Recent Breast-nonmalignant Pathology books
Atkins: 2012
Aydiner: 2015
Bhargava: 2015
Cangiarella: 2013
Chinyama: 2014
Dabbs: 2016
Hoda: 2014
Hoda: 2017
Lakhani: 2012
Lester: 2016
Mohsin: 2011
Sanders: 2014
Schnitt: 2012
Schnitt: 2012
Tot: 2014
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