Atypical Presentation of Granulosa Cell Tumor in an Adolescent: A Case Report

Background: Granulosa cell tumors (GCTs) frequently present with elevated levels of estrogen and inhibin. Most diagnoses in the pediatric and adolescent population are juvenile-type GCTs; adult-type GCTs in this population are rare. Case: We describe a 14-year-old female who presented with a large adnexal mass and clinical hyperandrogenism. Laboratory evaluation revealed elevated levels of free and total testosterone, low-normal estradiol, and mildly elevated alpha-fetoprotein (AFP). Other tumor markers, including inhibin, were within normal limits. Intraoperative assessment with unilateral oophorectomy, pathology, and imaging resulted in a diagnosis of a stage IA adult-type GCT. Summary and Conclusion: GCTs often result in elevated estrogen and inhibin B levels; however, this case demonstrates that non-classic elevations in testosterone and normal inhibin levels should not eliminate concern for a GCT, particularly in the setting of a large ovarian mass.