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Sex Cord-Stromal Tumors I

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 sex cord-stromal tumors 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.
5. Selected review articles as mentioned in individual entities.

Adult Granulosa Cell Tumor

Juvenile Granulosa Cell Tumor

Fibrosarcoma of Ovary

Sclerosing Stromal Tumor

Signet Ring Cell Stromal Tumor

Ovarian Myxoma

Sertoli-Leydig Cell Tumor of Ovary

Sex Cord Tumor with Annular Tubules