Juvenile granulosa cell tumor of the ovary presenting with pleural effusion and ascites

Juvenile granulosa cell tumor (GCT) is a rare tumor, and the majority (90%) are reported in the prepubertal or under-30-year age group, in contrast to the adult type, which is more common in the fifth decade. On histopathological examination, juvenile GCTs are distinct from the adult type of GCT, and have a lower risk for late recurrences than the latter. Being solid tumors, they may be associated with ascites and pleural effusion (Meigs’ syndrome), which resolve after surgical removal of the tumor. Tumor markers for GCT are still investigational (inhibin) and of not much use in making a preoperative diagnosis, unlike in the case of germ cell tumors. In most of the reports about the initial surgical management of GCT, retroperitoneal lymph node sampling was not performed, and it was not done in the patient we report here. However, lymph node sampling is advocated for complete staging of these tumors, as a significant number of recurrences are reported in the retroperitoneum, as well as in incompletely staged patients. In the present patient, because of the association of Meigs’ syndrome, a preoperative diagnosis of benign tumors such as fibroma/thecoma was also considered. We report this rare tumor with an aim of reviewing the diagnosis and management from the reported literature.