Breast – Malignant, Males, Children - Glycogen rich (clear cell) carcinoma

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Breast-malignant, males, children

Glycogen rich (clear cell) carcinoma

Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.

Reviewer: Daniel Visscher, M.D., University of Michigan Hospitals, February 2009 (see Reviewers page)

Revised: 26 September 2009

Last major update: September 2009

Definition

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● Breast carcinoma in which at least 90% of the neoplastic cells have abundant clear cytoplasm due to glycogen

● First described in 1981 (Cancer 1981;48:2003)

● Rare; 1-3% of breast carcinomas

● May be a variant of apocrine carcinoma

Treatment and prognosis

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● Prognosis similar to invasive ductal carcinoma (AJSP 1995;19:904)

Xray images

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Mammogram shows circumscribed mass

Case reports

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● 33 year old woman whose tumor had neuroendocrine features (Pathologica 2001;93:676)

● 45 year old woman with solid papillary tumor (J Clin Pathol 2003;56:552)

● 59 year old woman (World J Surg Oncol 2008;6:44)

● 61 year old woman (Archives 2003;127:1629)

Gross images

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Solid papillary pattern within cystically dilated duct (Fig 1A)

Microscopic description

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● Solid or solid / papillary patterns of large clear cells with distinct cell borders containing glycogen in 90% or more cells

● Often associated with intraductal component of varied type

● Often has apocrine features

● Cells have clear to granular cytoplasm

● May have scant intracellular mucin (Histopathology 1987;11:857)

● No cytoplasmic vacuoles

Micro images

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Tumor cells with distinct borders Resembles renal clear cell carcinoma

and clear cytoplasm

Tumor cells contain abundant cytoplasmic glycogen

Fig 1/2: Infiltrating nests of atypical cells with Polygonal cells with distinct cell borders,

abundant clear cytoplasm clear cytoplasm and prominent nucleoli (Fig 1B/1C)

Fig 3: PAS+ granules

Fig 4: PAS staining is removed by diastase

CK8/18+

AFIP images

Sheets of polygonal cells with distinct borders, Clear cells surround a small group of

clear cytoplasm and small nuclei, also tumor cells with amphophilic cytoplasm

lymphocytes and plasma cells (arrows)

PAS+ dark granules are due to glycogen, and are abolished by diastase

Cytology description

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● Hypercellular with tumor cells in loosely cohesive syncytial groups and some single cells

● Most tumor cells have abundant, finely granular eosinophilic cytoplasm or foamy to clear cytoplasm with well defined cytoplasmic membranes and moderate/marked nuclear pleomorphism with central round/oval nuclei containing prominent nucleoli (Acta Cytol 2008;52:65)

● PAS staining may be helpful (J Med Invest 2002;49:193)

Positive stains

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● PAS-diastase sensitive (glycogen)

Negative stains

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● CK20, lipid stains (done on fresh/frozen tissue)

Electron microscopy

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● Non-membrane bound glycogen and empty glycogen lakes

● Tight junctions between tumor cells, immature desmosomes, occasional short microvilli (AJSP 1986;10:553)

Differential diagnosis

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● Clear cell “sugar” tumor - positive for melanocytic markers (AJSP 2002;26:670)

● Lipid-rich carcinoma

● Apocrine carcinoma

● Secretory carcinoma - younger patients, low grade histology

● Myoepithelial lesions

Additional references

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● Hum Path 1991;22:81, Stanford University

End of Breast – Malignant, Males, Children > Glycogen rich (clear cell) carcinoma

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