ESPE Abstracts

Introduction: Granulosa cell tumors (GSTs) of ovary are very rare, usually large gonadic neoplasms arising from the mesenchyme and sex cords. The main clinical appearance of the juvenile GCTs is represented of signs of excessive estrogen (Precocious puberty - 75% of cases) or testosterone secretion (rare).

Aim: To present the different clinical presentation and outcome in a case series of a rare tumor patients.

Cases presentation: Case 1 16 years-old-girl, with complete pubertal development, hirsutism and acne was admitted for secondary amenorrhea. Hormonal assessment confirms the mild hyperandrogenism, but also undetectable gonadotrophins with high estradiol (258 pg/mL) and high CA 125. Imagistic evaluation shows a heterogenous lesion of 11/8/4 cm, with a thin capsule and bridging vessel sign on the right ovary for which she undergone surgery. Histopathology (HP) and immunohistochemistry (IHC) confirm the diagnosis of a juvenile GST, and poorly differentiated Sertoli-Leydig tumor, inhibin positive, Ki67 40%. Postsurgical outcome was favorable, with normalized estrogen secretion and negative inhibin B, but she remains under observation.

Case 2: 5.7 years-old-girl, admitted for precocious telarche, with hyperestrogenism (31 pg/mL) and undetectable gonadotrophins, high inhibin B (372 pg/mL), negative beta-HCG and alfa-fetoprotein. Pelvic MRI appearance consists of a multi-cystic and multi-septal 3.7/1.8/2 cm mass, with a thin clear capsule, located on the right ovary. Right ovariectomy was performed and a sex cord tumor with annular tubules was diagnosed (inhibin positive; Ki67 8%). Postsurgical outcome was persistent favorable at 1.8 years – clinical and morphological regression of the puberty signs, along with prepubertal gonadal axis, undetectable inhibin B and no imagistic evidence of recurrence.

Case 3: 10.5 years-old-girl admitted for prolonged menometrorrhagia and abdominal pain, was diagnosed with a well-defined mass of 16.4/8 cm, with liquid and solid areas on the CT-scan, with positive tumoral markers (CA 125 88 U/mL). Right adnexectomy with tumoral excision, followed by HP and IHC examination established the diagnosis of a juvenile GST, pT1a/pNx/pMx, positive for inhibin; adjuvant chemotherapy was performed due to high values of inhibin B. 1.2 years after the initial diagnosis biochemical (inhibin B 243 pg/mL) and morphological (12.7/9.5/10.5 cm, similar to the initial tumor) recurrence occurred; surgical treatment is incoming.

Discussion: The low frequency, variable manifestations and the absence of a specific tumoral markers may delay the diagnosis in case of a GSTs. Surgical removal is mandatory, and the prognosis depends on the initial disease staging; still, periodic long-term evaluation in needed.