Pure sertoli cell tumor of the ovary, a rare cause of isosexual pseudoprecocious puberty in a two year old girl

ABSTRACT

A case of a two and a half year old girl with a 6 month history of isosexual precocious puberty (thelarche Tanner stage 2 breasts and menarche) secondary to an estrogenizing pure Sertoli cell tumor of the left ovary is presented. Pre-operative tumor markers AFP, BhCG and CA-125 revealed normal results. Transabdominal ultrasound with color flow mapping and Doppler interrogation revealed a solid left ovarian mass probably malignant. The patient underwent exploratory laparotomy, left salpingooophorectomy and frozen section revealing sex cord stromal tumor. Paraffin sections stained with Hematoxylin and Eosin revealed a benign Sertoli cell tumor. Immunostains of the tumor reacted positively for inhibin, calretenin and cytokeratin, but negative for epithelial membrane antigen. Total serum estradiol, prolactin, TSH and LH were elevated prior to surgical operation, with LH and prolactin substantially decreasing four weeks later into the normal prepubertal range. TSH and estradiol levels however have remained very slightly elevated. Serum FSH was at prepubertal levels. Breasts size had likewise regressed to prepubertal size four weeks postoperatively, and the menses never recurred. This is the youngest reported occurrence of this rare sex cord stromal neoplasm in the last 10 years in our institution. The prognosis of this extremely rare tumor presenting at this early juvenile stage is uncertain, and it is recommended to keep a close follow-up and regular endocrinologic investigation until prepubertal values are attained.