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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

Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.

 

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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