46,XX male syndrome with mullerianosis of the urinary bladder: A new clinical entity.

Mullerianosis of the urinary bladder is a rare entity characterized by the presence of an admixture of at least two types of mullerian tissue in the muscularis propria of the bladder. We report the case of a 43-year-old male patient presented for urological evaluation due to episodes of total gross hematuria. Physical examination revealed ambiguous external genitalia. Abdomiopelvic CT scan revealed a tumor at the level of the bladder floor. Transurethral resection of the bladder lesions was done and histopathological studie confirmed the diagnosis of mullerianosis. Karyotype analysis showed a 46 XX male syndrome.