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Breast malignant, males, children

Carcinoma subtypes

Glycogen rich (clear cell) carcinoma

Reviewer: Monika Roychowdhury, M.D., (see Reviewers page)
Revised: 10 October 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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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
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● Prognosis similar to invasive ductal carcinoma (Am J Surg Pathol 1995;19:904)

Radiology 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)
● 55 year old woman (Case Rep Oncol 2011;4:452)
● 59 year old woman (World J Surg Oncol 2008;6:44)
● 61 year old woman (Arch Med Lab Pathol 2003;127:1629)
● Intraductal lipid rich carcinoma with component of glycogen rich carcinoma (J Breast Cancer 2012;15:135)

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

Micro 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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Various images

       
Intraductal lipid-rich carcinoma with component of glycogen-rich carcinoma


Tumor cells with distinct borders and clear cytoplasm


Resembles renal clear cell carcinoma

   
Tumor cells contain abundant cytoplasmic glycogen


Fig 1/2: Infiltrating nests of atypical cells with abundant clear cytoplasm
Fig 3: PAS+ granules
Fig 4: PAS staining is removed by diastase


Polygonal cells with distinct cell borders,
clear cytoplasm and prominent nucleoli (Fig 1B/1C)


CK8/18+

AFIP images:

Sheets of polygonal cells with distinct borders, clear cytoplasm and
small nuclei, also lymphocytes and plasma cells (arrows)


Clear cells surround a small group of tumor cells with amphophilic cytoplasm


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 microscopic description
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● Non-membrane bound glycogen and empty glycogen lakes
● Tight junctions between tumor cells, immature desmosomes, occasional short microvilli (Am J Surg Pathol 1986;10:553)

Differential diagnosis
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● Apocrine carcinoma
● Clear cell “sugar” tumor: positive for melanocytic markers (Am J Surg Pathol 2002;26:670)
● Lipid-rich carcinoma: positive for lipid stains, negative for glycogen
● Myoepithelial lesions
● Secretory carcinoma: younger patients, low grade histology

Additional references
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● Hum Pathol 1991;22:81, Stanford University

End of Breast malignant, males, children > Carcinoma subtypes > Glycogen rich (clear cell) carcinoma

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