A 44-year old phenotypic female, married with primary amenorrhea presented with recurrent urinary tract infection and an incidental finding of a pelvo-abdomlnal mass.Ultrasonogram with doppler studies showed a huge pelvo-abdominal mass with rich intratumoral and peripheral color flow and low resistance index. No uterus or cervix was demonstrated. Tumor marker, CA-125 level, was elevated. Exploratory laparotomy confirmed the absence of the uterus and fallopian tubes. The left "ovary" was converted to a 16cm x 15cm x 12cm predominantly solid encapsulated mass. The capsule was smooth with a point of rupture measuring approximately 1 cm in diameter, exuding fresh blood. The right "ovary" was grossly normal measuring 3cm x 2cm x 2cm. Bilateral "oophorectomy", peritoneal fluid cytology, infracolic omentectomy with frozen section, were carried out. Histopathology of the left pelvic mass revealed testicular seminoma, while the right mass was consistent with an atrophic testis. Peritoneal fluid was positlve for malignant cells. Referral to a gynecologic oncologist was done for adjuvant radiation therapy. With the late realization of her being genetically male,psychological therapy and genetic counseling were advised.

Human ; Female ; Adult ; Fallopian Tubes ; Ovary ; Frozen Sections ; Seminoma ; Cervix Uteri ; Ascitic Fluid ; Amenorrhea ; Laparotomy ; Uterus ; Ovariectomy ; Urinary Tract Infections