(age range 7mo-14 yrs) Juvenile granulosa cell tumor of the ovary: a clinicopathologic study

abstract

Study Objective

To report on the clinical characteristics and outcome of pediatric patients with juvenile granulosa cell tumor (JGCT) of the ovary.

Design, Setting, and Participants

Patients with histopathologically confirmed ovarian JGCT diagnosed between 1990 and 2016 were identified. Data on the clinical presentation, surgical management, oncologic management, laboratory investigation, follow-up, and outcome were collected. Tumor was staged according to the International Federation of Gynecology and Obstetrics (FIGO) criteria.

Results

Eight patients were diagnosed with ovarian JGCT during the study period. The median age at presentation was 3 years (range 0.7 – 14 years). Precocious puberty was the presenting symptom in all five prepubertal children; abdominal distension due to mass effect was the presenting symptom in three children greater than 9 years of age. In patients who had preoperative serologic testing, estradiol (n=3) and inhibin (n=3) were elevated. Five patients had stage I disease, and three had stage III. All stage I patients underwent salpingo-oophorectomy as the only treatment. Stage III patients received adjuvant chemotherapy. After a median follow-up of 6.2 years, six patients (75%) were alive without evidence of disease. One stage I patient with germline p53 mutation and PTEN mutation, died due to subsequent liposarcoma. One patient with stage IIIB disease developed recurrence detected by elevated inhibin serum level, and died due to progressive disease despite receiving multiple chemotherapy regimens.

Conclusion

JGCT has a favorable prognosis in patients with stage I disease following surgical resection alone. Adjuvant chemotherapy may be indicated in patients with higher stage tumors.

Key Words

• Juvenile granulosa cell tumor;
• pathology;
• inhibin B;
• chemotherapy