Signet ring cell carcinoma (SRCC) of the breast is a rare tumor and it is classified by World Health Organization in 2003 classification under ‘mucin producing carcinomas’. Pure form of SRCC breast is an extremely rare entity and very few cases have been reported in literature so far. We present a case of pure primary SRCC of the breast in a 70-year-old female, which was diagnosed on fine needle aspiration cytology. Cytological features generally show cellular smears with tumor cells showing eccentrically placed large, irregular nuclei showing indentations at places with cytoplasmic vacuoles. This case is being presented in view of its characteristic cytological features and its rarity.
Familial cylindromatosis (FC) is an autosomal dominant disorder with apparently complete penetrance, but variable expression. There is an increasing evidence that FC is clinically, genetically, and histologically heterogeneous disorder as the simultaneous occurrence of cylindromas and other tumors of skin appendages within the affected individuals and families. The presence of multiple scalp cylindromas is often associated with autosomal dominant Brooke-Spielger syndrome, a condition in which there are co-existent facial trichoepitheliomas and spiradenomas. We present here a case of multiple cylindromatosis in a family affecting many members successively.
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