Stage I juvenile granulosa cell tumors of the ovary: A multicentre analysis from the MITO-9 study

Eur J Surg Oncol. 2021 Jul;47(7):1705-1709. doi: 10.1016/j.ejso.2021.02.003. Epub 2021 Feb 9.

Abstract

Objective: Juvenile type granulosa cell tumor (JGCTs) are extremely rare, mainly diagnosed in young women and pre-pubertal girls at stage I disease. Literature is scanty and guidelines regarding the optimal management are still controversial. The aim of this study is to add on the experience of the MITO group (Multicenter Italian Trials in Ovarian Cancer).

Methods: Clinicopathological data from patients with stage I JGCTs were retrospectively collected. Descriptive statistics were used to characterize the patient population. Clinicopathological features and treatment variables were evaluated for association with relapse.

Results: Seventeen patients were identified. Surgical approach was laparoscopic and open for 7 (41%) and 10 (59%) patients, respectively. Fertility sparing surgery (FSS) was performed in 15 patients (88%): unilateral salpingo-oophorectomy (USO) in 11 patients, cystectomy with subsequent USO in 2 patients and cystectomy alone in the remaining 2. Adjuvant chemotherapy was given in 2 cases. After a median follow up time of 80 months, no recurrences were registered.

Conclusions: Given the available data, minimally invasive surgery is safe in stage I JGCTs. Because of the good prognosis and of the young age of patients, FSS can be chosen in most of the cases. The role of cystectomy deserves further validation. The need of adjuvant chemotherapy in stage I disease is still unclear, even if available data does not seem to support treatment over surveillance.

Keywords: Juvenile-type ovarian granulosa cell tumors; Ovarian cancer; Sex cord stromal tumors.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Female
  • Granulosa Cell Tumor / pathology
  • Granulosa Cell Tumor / surgery*
  • Humans
  • Italy
  • Minimally Invasive Surgical Procedures*
  • Neoplasm Staging
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / surgery*
  • Ovariectomy
  • Prognosis
  • Retrospective Studies
  • Salpingo-oophorectomy