Juvenile Granulosa Cell Tumors of the Ovary A Clinicopathologic Study of 7 Cases and a Review of the Literature

Objective: To explore the clinical and pathologic features of ovarian juvenile granulosa cell tumors ( JGCTs). Methods: Clinical data, histopathologic observations, immunohistochemical results, FOXL2 mutation status and follow-up information of 7 JGCT cases were studied Results: The patients most commonly presented with abdominal distension and pain (5 cases), followed by precocious puberty (1 case) and a pelvic mass (1 case). Six patients had stage I disease, and 1 had stage IV disease. The microscopic examinations typically showed lobular growth punctuated by variably sized and shaped follicles Rare features included a reticular-cystic appearance mimicking a yolk sac tumor (2 cases), a lobular appearance similar to a sclerosing stromal tumor (1 case), strands and cords (1 case), pseudopapillary appearance (2 cases), spindle cell appearance ( 1 case), microcvstic appearance (1 case), hobnail cells (1 case), and rhabdomyoid cells (1 case). No FOXL2 mutation was encountered After a median follow-up of 53 months only 1 patient with a strongly diffuse TP53-positive tumor died of the disease, and 2 successfully had babies. Conclusions: JGCT is a rare neoplasm with a wide morphologic spectrum and is easily confused with other tumors. Familiarity with the characteristics, rare atypical appearances and immunohistochemical results may aid in obtaining a correct diagnosis.