Juvenile granulosa cell tumor: An interim report from the international ovarian and testicular stromal tumor (OTST) registry.

Abstract

6064 Background: Juvenile granulosa cell tumors (JGCT) are rare sex cord-stromal tumors which occur primarily in children and adolescents. Methods: All individuals or proxy caregivers provided informed consent/assent for participation in the International OTST Registry. Clinical data was collected. When available, pathology was centrally reviewed. Staging was evaluated using the International Federation of Gynecology and Obstetrics (FIGO) classification. Kaplan-Meier survival analyses and exact permutation tests were performed. Results: Forty-two individuals with ovarian JGCT were enrolled. Median age at diagnosis was 9 years (range 0-27). Most individuals had Stage I disease (Stage Ia=16; Stage Ib=1; Stage Ic=16). Seven individuals presented with higher stage (Stage II=2; Stage III=5). Stage was unknown for 2 individuals. Three-year overall survival (OS) was 88% (CI 77%, 100%) and event-free survival (EFS) was 69% (CI 54%, 88%). At median follow-up time of 25 months (range 0-416), 9 patients (Stage Ia=1, Stage Ic=5, Stage III=3) had recurrent disease. Use of post-operative adjuvant chemotherapy varied by stage and timing of rupture. Of those with Stage Ic JGCT, 2/7 with preoperative rupture and 3/9 with intraoperative rupture recurred. Among individuals with recurrence, median time to recurrence was 11.5 months (range 3-19). Four of 9 individuals with recurrence survived (no evidence of disease n=2; alive with disease n=2). All individuals who died presented with extrapelvic recurrence. Median time from recurrence to death was 10 months (range 2-53). In individuals with recurrence, advanced stage at diagnosis (HR 5.1; p-value 0.087) and recurrence outside the tumor bed (HR Infinity; p-value 0.048) were associated with inferior OS. Three-year OS for individuals with recurrence was 57% (CI 30%, 100%). Conclusions: Low stage JGCT is associated with a favorable prognosis, however, recurrence is associated with lower survival rate. Within this series, recurrences presented within 2 years of diagnosis. Novel strategies are needed to address recurrent and extrapelvic disease.

Contemporary Updates on Sex Cord–stromal Tumors of the Testis

“…9,58 In contrast, ovarian JGCTs affect young prepubertal children with a median age of 7 to 8 years but may also arise in postpubertal women, and a subset behaves aggressively. 62,63 Moreover, a significant proportion of ovarian JGCTs is associated with precocious pseudopuberty, whereas most testicular JGCTs are hormonally quiescent. 62 Molecular pathogenesis of ovarian JGCTs is characterized by the presence of GNAS and AKT1 variants.…”

“…Testicular JGCTs are invariably indolent and do not recur after complete surgical excision 9,58 . In contrast, ovarian JGCTs affect young prepubertal children with a median age of 7 to 8 years but may also arise in postpubertal women, and a subset behaves aggressively 62,63 . Moreover, a significant proportion of ovarian JGCTs is associated with precocious pseudopuberty, whereas most testicular JGCTs are hormonally quiescent 62 .…”

Complete restoration of fertility in a patient treated for androgen-secreting granulosa cell tumor- Case report

“…Two non-typical forms of granulosa cell tumor are juvenile and cystic types. Both occur in children and young adults ( Harris et al ., 2020 ). The juvenile type of this tumor occurs before puberty and causes precocious pseudopuberty.…”