Oncologic and fertility outcomes in patients with juvenile granulosa cell tumor - a retrospective single centre analysis.

Abstract

Background: Granulosa cell tumors (GCTs) are rare neoplasia that account for less than 5 % of all the ovarian tumors. Juvenile GCT histotype is generally observed in adolescent and young women, representing a very rare disease, so only a paucity of data are present in literature. The aim of this study is to analyse the oncologic and fertility outcome in our case series of juvenile GCTs.

Methods: Clinicopathological data were retrospectively collected and analysed from a cohort of 30 patients ovarian juvenile GCTs treated at IRCCS San Gerardo dei Tintori Hospital, Monza, between 1980 and 2024.

Results: The median age of disease onset was 21.5. Among patients enrolled in the study, 80.0 % (24/30) were stage I (16/26, 1/26 and 7/26 of stage IA, IB and IC, respectively), 6.7 % (2/30) were stage II and 13.3 % stage III (4/30). In 86.7 % (26/30) of patients, a fertility-sparing surgery was carried out, while 13.3.% (4/30) of patients underwent radical surgery. Adjuvant chemotherapy was administered in 20.0 % (6/30) of cases, while 80.0 % (24/30) were followed only with surveillance. Three patients in thirty (10.0 %) relapsed and died of disease despite multi-therapeutic approaches. All of them had advanced stages of disease at time of diagnosis.

Conclusions: Juvenile GCT appears to have a good prognosis at stage I disease. However, advanced stage represents a hard challenge for clinicians, showing a high rate of relapse and mortality.