A rare encounter with juvenile granulosa cell tumor: diagnosis, management, and outcome

Abstract

Juvenile granulosa cell tumor (JGCT) is a rare ovarian sex cord-stromal tumor, accounting for less than 5% of all granulosa cell tumors and primarily affecting prepubescent girls and young women. Due to its rarity, clinical presentation, optimal management, and long-term outcomes remain areas of ongoing investigation. We report the case of a 19-year-old nulliparous female who presented with intermittent lower abdominal pain and bloating for eight months, with no menstrual irregularities or signs of virilization. Clinical examination revealed a firm, 20-week-sized pelvic mass, and imaging studies suggested a large multiseptated right abdominopelvic mass. Patient underwent fertility-sparing staging laparotomy, and histopathology confirmed JGCT with micro metastasis in the omentum (pT3aN0). Immunohistochemistry showed positivity for inhibin, PR, calretinin, and WT1. The patient underwent four cycles of adjuvant paclitaxel and carboplatin chemotherapy and has remained in remission for one year and under three-monthly surveillance. This case highlights the importance of early recognition, comprehensive surgical staging, and appropriate adjuvant therapy in managing JGCT. Given its potential for aggressive behavior and recurrence, long-term follow-up is essential. This report underscores the need for individualized treatment approaches in rare ovarian malignancies.