LETTER TO EDITOR
Year : 2010 | Volume
: 53 | Issue : 2 | Page : 374--375
A case report of glycogen-rich clear cell carcinoma of breast
Subbanna Rekha Thondavadi, Jayashree Krishnamurthy, Vimala Manjunath Gubbanna
Department of Pathology, J.S.S Medical College, Mysore, India
Subbanna Rekha Thondavadi
#818, 13th main, 4th stage, T.K. Extension, Mysore - 570 009
|How to cite this article:|
Thondavadi SR, Krishnamurthy J, Gubbanna VM. A case report of glycogen-rich clear cell carcinoma of breast.Indian J Pathol Microbiol 2010;53:374-375
|How to cite this URL:|
Thondavadi SR, Krishnamurthy J, Gubbanna VM. A case report of glycogen-rich clear cell carcinoma of breast. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Aug 4 ];53:374-375
Available from: http://www.ijpmonline.org/text.asp?2010/53/2/374/64289
Glycogen-rich clear cell carcinoma (GRCC) of breast, a rare primary malignant lesion, was first described by Hull in 1981.  The clinicopathological features and prognosis is still unclear. The clear cell component being more than 90% as a criterion, Toikkanen  reported the incidence to be 1.4%, as opposed to 2.7% by Kuroda.  The prognosis was variable, with majority of them being reported as aggressive while there were a few cases with favorable prognosis. 
A 52-year female presented with a solitary, painless mass measuring 6 x 7 cm in the right breast since three months. On examination the mass was hard in consistency and mobile. Fine needle aspiration cytology was reported as suspicious of malignant lesion for which patient underwent excisional biopsy, followed by modified radical mastectomy (MRM).
The excisional biopsy of the mass showed a malignant tumor composed of ducts of varying sizes filled with tumor cells arranged in comedo, cribriform and solid patterns [Figure 1]. The individual cells were uniform, large, round to polygonal having distinct cell margins. The cells had abundant and clear cytoplasm with mildly pleomorphic, hyperchromatic nucleus. The tumour cells were seen infiltrating the stroma in sheets, groups, cords and in singles, which totally comprised more than 90% of the tumor component. Histochemically the tumour cells were Periodic Acid Schiff (PAS) positive and diastase labile [Figure 2], estrogen receptor positive (score 6), but negative for progesterone receptor and C erbB2. Keratin AE1/AE3, EMA and cytokeratin that suggest GRCC, while smooth muscle actin and S-100 that rules out myoepithelial involvement were not performed due to nonavailability of the facility, and the diagnosis was made on the morphology and diastase labile PAS positivity. Metastatic renal cell carcinoma was ruled out by an extensive search for the primary in the kidneys. The MRM specimen showed no residual malignancy in the breast tissue but three out of six axillary lymph nodes had metastasis.
Glycogen-rich clear cell carcinoma is a rare primary malignant neoplasm and has to be differentiated from signet-ring carcinoma, lipid-rich carcinoma, apocrine carcinoma and secretory carcinoma, which also consist of clear cell morphology with the help of histochemical examination and immunohistochemistry (IHC). The GRCC have cytoplasmic granular PAS positive and diastase labile material, whereas signet-ring carcinoma is PAS positive and diastase resistant.  Lipid-rich carcinoma is Oil Red O and Sudan-black positive. Secretory carcinoma is again PAS positive but diastase resistant and mucicarmine may or may not be positive. Unlike GRCC, apocrine carcinoma is gross cystic disease fluid protein-15 positive.
The nature of these cells requires multicentric studies comprising large series. Hull  has described the light and electron microscopic features and has reported that the clear cells have features similar to those of the fetal breast and to other clear cell carcinoma arising elsewhere in the body. Di Tommaso  has reported a case of GRCC with neuroendocrine differentiation. Staining for chromogranin was positive in some cells and for synaptophysin in most cells, indicating a degree of neuroendocrine activity of the tumor  . Markopoulos have extensively analysed with various IHC stains and have reported as positive for PAS staining erased by diastase pre-treatment. 
J.S.S.Medical College, Mysore.
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