A Systematic Review on Giant Ovarian Sex Cord Stromal Tumors Presenting as Massive Abdominopelvic Masses

Abstract

Background: Ovarian sex cord stromal tumors (SCSTs) are rare neoplasms accounting for 5-8% of all ovarian malignancies. Giant variants (>10 cm) are exceptional and mimic epithelial tumors clinically and radiologically. Aim and Objective: To systematically review the clinical, diagnostic, and therapeutic aspects of giant ovarian SCSTs. Methods: A systematic search (PubMed, Scopus, Google Scholar, 2015-2025) used keywords “giant ovarian sex cord stromal tumor”, “granulosa cell tumor”, “fibrothecoma”, and “Sertoli-Leydig cell tumor”. Articles reporting histologically confirmed tumors >10 cm were analyzed. Results: Forty-two studies comprising 67 patients were included. Mean age was 47 years (range 14-78). The commonest histologic type was adult granulosa cell tumor (53%), followed by fibrothecoma (31%) and Sertoli-Leydig tumor (16%). Average size was 25.4 cm. Abdominal distension and pain were the predominant symptoms (85% and 72%), with hormonal effects in 22%. Radiologically, 62% were misdiagnosed as epithelial tumors. Surgery was curative in most cases; recurrence occurred in 9%, mainly granulosa variants. Conclusion: Giant ovarian SCSTs are rare but largely benign. Early diagnosis, surgical excision, and long-term follow-up ensure favorable prognosis.