Image Description
Gross Pathology: Ovarian steroid cell tumors are well-circumscribed tumors measuring around 8 cm on average (range 1.2 cm to >40 cm). Larger tumors are more likely to be malignant. Most are solid, but occasional cases may be partially cystic or predominantly cystic. The cut surface shows bright yellow, orange, or yellow-brown lobules separated by fibrous septa. Areas of hemorrhage or necrosis may be present and are frequently associated with malignant behavior. Stromal luteomas, which are morphologically identical to steroid cell tumor, are by definition < 2cm in size and confined to the ovary.
Image courtesy of: Dr. Vijay Shankar S, Professor of Pathology, Bengaluru, India; used with permission.
About the Disease
Sex cord-stromal tumors of the ovary are a heterogenous group of relatively rare neoplasms arising from hormone-producing cells of ovarian cortical stroma. They make up approximately 8% of all ovarian tumors and are the most common ovarian tumors with endocrine manifestations. WHO Classification subdivides them into 3 main categories based on the predominant cell types. Pure stromal tumors have a mesenchymal appearance and include: fibroma, cellular fibroma, thecoma, luteinized thecoma with sclerosing peritonitis, sclerosing stromal tumor, microcystic stromal tumor, signet-ring stromal tumor, Leydig cell tumor, steroid cell tumor, and fibrosarcoma.Pure sex cord tumors have an epithelioid appearance and include: adult granulosa cell tumor, juvenile granulosa cell tumor, Sertoli cell tumor, and sex cord tumor with annular tubules.Mixed sex cord-stromal tumors include Sertoli-Leydig cell tumor, sex cord-stromal tumor, NOS, and gynandroblastoma.The most common are tumors in fibroma-thecoma group (80-85%) followed by granulosa cell tumors (10-15%). The remainder are quite rare. Most of the clinically malignant tumors are granulosa cell tumors. References:1. Mutter G. L.& Prat J (2014). Pathology of the Female Reproductive Tract - 3rd Edition. Churchill Livingstone Elsevier.2. WHO Classification of Tumors. Female Genital Tumors. 5th Edition, 2020; IARC, Lyon, France.3. Nucci MR, Parra-Herran C (2021). Gynecologic Pathology. 2nd Edition. Philadelphia, PA. Elsevier.4. Goldblum, J. R. et al (2018). Rosai and Ackerman's Surgical Pathology - Eleventh Edition. Philadelphia, PA. Elsevier.