Clear cell carcinoma of Mullerian origin in the urinary bladder: A rare entity

Mullerian neoplasms of the urinary system are rare but complex tumor-like lesions. The identification of the Mullerian neoplasm is crucial for patient management owing to its etiology, natural history, and prognosis. We present a case of a 42-year-old female with a history of three lower segment cesarean sections presenting with complaints of dysmenorrhea and suprapubic pain with no history of hematuria or any urinary symptoms. Magnetic resonance imaging revealed a 2 cm×2 cm exophytic lesion suspicious of being either a bladder lesion or an endometrial lesion infiltrating the urinary bladder. Cystoscopy and transurethral biopsy of this suspicious bladder tumor revealed a malignant tumor with papillary and tubulocystic architecture. Based on the overall histomorphological and immunohistochemical features, a diagnosis of clear cell carcinoma of Mullerian origin was made, and the patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy and partial cystectomy. The patient was kept on regular surveillance and showed no signs of recurrence at the one-year follow-up. Clear cell carcinoma of the bladder of Mullerian origin is a rare entity and is established on histopathology. Prompt diagnosis and a multidisciplinary approach are indispensable for management. Level of evidence: Level 4.