The many faces of ovarian sex cord stromal tumors: a clinicopathological analysis at a tertiary cancer center in North India

Abstract Background Ovarian sex cord-stromal tumors (OSCSTs) represent a rare and heterogeneous group of neoplasms originating from the ovarian sex cords and stroma. These tumors account for approximately 5–7% of all ovarian cancers. They encompass a diverse array of histopathological subtypes and exhibit significant histomorphological diversity, which underpins their varied clinical presentations and biological behaviors. The authors embarked on this study to chronicle the clinical presentation, the myriad histologic miens of OSCSTs, the associated diagnostic pitfalls and utilitarian pearls, treatment strategies, and outcomes of these patients. Methods A retrospective analysis was conducted on patients diagnosed with OSCSTs at a tertiary cancer care center in North India from July 2019 to June 2023, over a period of 4 years, with due approval from the Institutional Ethics Committee. Results Thirty-five patients with OSCSTs were identified, affecting 88.6% adults and 11.4% children or adolescents. The predominant symptoms were abdominal pain (65.7%) and distension (42.8%). Bilateral ovarian involvement was ascertained in 42.8% of cases, and metastasis was detected in 34%, predominantly in the omentum/peritoneum. The most common histological type was adult granulosa cell tumors, followed by fibromas, Sertoli-Leydig cell tumors, juvenile granulosa cell tumors, and steroid cell tumors. These tumors manifested a confounding variety of histomorphologic facets, resulting in consideration of wide-ranging differential diagnoses. Early-stage (stage I) disease was diagnosed in 57.7% of cases, while 42.3% presented at advanced stages (stages III and IV). There were no significant differences in histopathological features across the stages; however, higher stages correlated significantly with disease progression. Over a median follow-up period of 36 months, the overall 5-year survival rate was 95.5% ± 4.4%, with a mean survival time of 66.2 months. Conclusion OSCSTs exhibit a wide range of histopathological features and predominantly present at an early stage, with a high overall 5-year survival rate. However, advanced-stage OSCSTs can behave erratically clinically, necessitating the prediction of such occurrences. Pathologists must be conversant with the histologic diversity and be vigilant in avoiding misdiagnosis. The role of a multidisciplinary focus on developing efficacious models for risk assessment stratification cannot be overemphasized.