Retrospective analysis of clinicopathological characteristics of 19 ovarian juvenile granulosa cell tumor cases

Aim: To describe the clinical and pathological characteristics, diagnosis, treatment, and outcomes of juvenile granulosa cell tumor (JGCT).

Methods: We retrospectively analyzed the data of 19 patients with histopathologically confirmed juvenile granulosa cell tumors treated in two medical centers in Wuhan city of China between 1999 and 2019.

Results: Totally, 19 patients were included during the period. The median age at diagnosis was 8.25 years (range, 0.25-28 years). The most common clinical presentation was abdominal pain, five out of 10 prepubertal children presented with precocious puberty. Three patients underwent radical surgery (including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic and para-aortic lymphadenectomy), the other 16 patients had fertility-sparing surgery (cystectomy or ipsilateral salpingo-oophorectomy with or without omentectomy and lymphadenectomy). Eighteen patients had the international federation of gynecology and obstetrics (FIGO) stage I tumors, one patient had FIGO stage II disease. Twelve patients received postoperative adjuvant chemotherapy. The median follow-up time from the time of diagnosis was 35 months (range, 13-250 months). One patient experienced relapse and died of the disease 32 months after the initial diagnosis. Eighteen patients were alive and had not experienced recurrence during the follow-up period. The reproductive age patients that received fertility-sparing surgery had regular menstruation.

Conclusions: A majority of JGCTs are diagnosed as FIGO stage I tumors and have favorable clinical outcomes. Adjuvant chemotherapy seems to improve outcomes for patients with advanced-stage JGCTs; however, the value of chemotherapy in stage Ic patients is still unknown. Fertility sparing surgery should be considered in young patients who wish to bear children.