Androgenic Adult Granulosa Cell Tumor in a 13-Year-Old Prepubertal Patient: A Case Report and Review of the Literature

Abstract

We report the clinicopathologic findings of an unusual case of adult granulosa cell tumor with androgenic manifestation in a 13-year-old prepubertal girl. The patient had never had a menstrual period and presented with a 1 year history of hirsutism. Physical examination was only remarkable for an increase in facial and abdominal hair, both with a male pattern of distribution. A pelvic ultrasound demonstrated a 6.0 cm right adnexal cyst. Plasma testosterone and 17-hydroxyprogesterone levels were elevated. The patient initially was treated with monophasic oral contraceptive pills for 3 months and owing to lack of response, she underwent an exploratory laparotomy in which a left ovarian tumor, 7.0 cm in greatest dimension, and a 6.5 cm right paratubal cyst were found. A wedge biopsy of the left ovary and subsequent left oophorectomy with right salpingectomy were performed. No gross evidence of disease outside the ovary was noted. Microscopic examination of the left ovarian tumor revealed the typical features of an adult granulosa cell tumor. No tumor was seen outside the ovary. Six days after surgery, plasma testosterone and 17-hydroxyprogesterone levels were in the normal range. Nine months postoperatively, the patient shows no evidence of disease. To our knowledge, this represents the first case of a prepubertal patient with an adult granulosa cell tumor with androgenic manifestations reported in the English literature.

The Multifaceted Granulosa Cell Tumours—Myths and Realities: A Review

“…The two forms differ with regard to histologic features and clinical behaviour [24]. AGCT has been reported in children [25]; however, less than 1% of these lesions occur in prepubertal girls [26]. Upto 90% of JGCTs are diagnosed in patients under the age of 30 with half of JGCT cases seen in less than 10 years of age [22, 27] and 10% occurring in infants less than one year [5].…”

Virilization persists in a woman with an androgen-secreting granulosa cell tumor

“…Some of these patients experienced virilization (2). In all reported cases with virilization, signs of hirsutism and virilization improved shortly after tumor resection (2)(3)(4)(5)(6)(7). This is the first case of persistent masculinization resulting from an androgen-secreting GC tumor, despite a long-term postoperative normalization of androgen levels.…”

Unusual Virilization in Girls with Juvenile Granulosa Cell Tumors of the Ovary Is Related to Intratumoral Aromatase Deficiency

“…Although hyperandrogenism in granulosa cell tumors of the ovary has been reported by several investigators [1,7,8,18,19,20,21,22], its pathophysiology has not been elucidated so far. We recently showed that low or absent expression of SOX9 along with aberrant expression of FOXL2 in granulosa cell tumors of the testis could be associated with a transdifferentiation of tumoral cells from male to female type [9].…”