Abstract

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• It is rare to see an adult presenting with exstrophy of the bladder. Malignant conversion in exstrophy occurs in 4%, with adenocarcinoma as the most common histopathology. We report the first case of metastatic high grade urothelial carcinoma with squamous and sarcomatoid differentiation arising from undiagnosed, closed bladder exstrophy in a female at advanced age with associated bilateral deep vein thrombosis and clot retention. The patient developed clinical progression of disease despite neoadjuvant gemcitabine-cisplatin and salvage (or palliative) radiotherapy. To the best of our knowledge, this is the first reported case of a primary urothelial malignancy in occult bladder exstrophy.