Primary clear-cell urothelial carcinoma of urinary bladder: a not-so-clear entity with review of literature

Abstract

Primary clear-cell urothelial carcinoma (CCUC) is an uncommon type of urothelial cancer with only 16 cases reported in published literature. Due to the rarity of the tumour, its clinical and prognostic values have not been clearly understood. We present one such rare clinical diagnosis in a 60-year- old man who underwent radical cystectomy (RC) with ileal conduit for urinary bladder cancer. Histopathology showed features of high-grade CCUC infiltrating the muscularis propria. Immunohistochemistry revealed diffuse immunopositivity of pan cytokeratin (CK), GATA3, P40, CK7 but was immunonegative for CD10 and vimentin. Our patient expired 4 months after diagnosis. CCUC has recently been included in the WHO 2016 classification of urothelial tumours. Most of the patients present with poor prognosis. Accurate diagnosis and recognition of this unusual variant are essential for better patient management and prognosis. Early RC seems to be the preferred way of management.