We report a case of juvenile granulosa cell tumor of the ovary in a 7.4 years old girl, who presented isosexual pseudo-precocious puberty of progressive outcome and palpable hypogastric mass. Hormonal measurements showed: oestradiol: 296pg/ml; 17-alpha-OH-progesterone: 20ng/ml; DHEAS: 27µg/dl; betahCG: 0.66mIU/ml; testosterone: 120ng/dl; progesterone: 3.4ng/ml; LH: 0.5mIU/ml; and FSH: 0.01mIU/ml not responsive to GnRH test (Relisorm®: 100µg iv). Abdominal images confirmed left ovarian mass. Unilateral salpingo-oophorectomy was performed with complete resection of a 656g and 14.5 x 10 x 7cm tumor restricted to the left ovary. The histologic analysis revealed a juvenile granulosa cell tumor. During the surgery blood from left ovary vein was obtained and steroids concentration were analyzed. Elevated levels of oestradiol (969pg/ml), progesterone (15.8ng/ml), 17-alpha-OH-progesterone (34ng/ml) and testosterone (158ng/dl) were found. Few days after the surgery, rapid regression of breast and pubic hair was observed, with return of steroid concentrations to the normal pre-pubertal levels. During 9 years of follow-up, normal pubertal development happened at an expected age, and no signs of tumor relapse were found.
Precocious puberty; Ovarian tumor; Juvenile granulosa cell tumor; Treatment; Outcome
