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ovarian sex cord-stromal tumors

Sunday 18 December 2005

Definition: Ovarian sex cord-stromal tumors are a heterogeneous group of benign or malignant tumors that develop from the cells surrounding the oocytes, including the cells that produce ovarian hormones (ie, the nongerm cell and nonepithelial components of the gonads).

Ovarian sex cord-stromal tumors are rare, comprising 5 to 8 percent of all primary ovarian neoplasms.

In contrast to epithelial ovarian cancer, most patients with malignant sex cord-stromal tumors are diagnosed with early-stage disease, the tumors are generally considered to be low-grade malignancies, and there is no known association with the BRCA1 or BRCA2 germline mutations and genetic predisposition to breast and ovarian cancer.

Types

- ovarian granulosa-stromal cell tumors

  • ovarian adult granulosa cell tumor (adult-type granulosa cell tumor)
  • ovarian juvenile granulosa cell tumor (juvenile type granulosa cell tumor)
  • ovarian thecoma-fibroma group (fibroma-thecoma tumor group)
  • ovarian fibroma and ovarian cellular fibroma
  • ovarian fibrosarcoma
  • ovarian stromal tumor with minor sex cord elements
  • ovarian sclerosing stromal tumor
  • ovarian signet-ring stromal tumor

- ovarian Sertoli-stromal cell tumors

  • steroid cell tumors
    • steroid cell tumors, NOS (not otherwise specified)
    • stromal luteoma
    • ovarian Leydig cell tumor
      • ovarian hilus cell tumor
      • Leydig cell tumor, non-hilar type

- ovarian mixed sex cord-stromal tumors
- ovarian unclassified sex cord-stromal tumors

See also

- Tumors

  • testicular tumors

Links

- Histopathology images on Medscape

References

- Sex cord stromal tumors of the ovary in children: A clinicopathological report from the Italian TREP project. Cecchetto G, Ferrari A, Bernini G, Alaggio R, Collini P, Virgone C, Terenziani M, Dall’igna P, Cozza R, Conte M, Bisogno G. Pediatr Blood Cancer. 2011 Jul 1;56(7):1062-7. PMID: 21488154

- Tsuji T, Catasus L, Prat J. Is loss of heterozygosity at 9q22.3 (PTCH gene) and 19p13.3 (STK11 gene) involved in the pathogenesis of ovarian stromal tumors? Hum Pathol. 2005 Jul;36(7):792-6. PMID: 16084949

- Miettinen M, Wahlstrom T, Virtanen I, Talerman A, Astengo-Osuna C. Cellular differentiation in ovarian sex-cord-stromal and germ-cell tumors studied with antibodies to intermediate-filament proteins. Am J Surg Pathol. 1985 Sep;9(9):640-51. PMID: 2996374


- Histopathology images on Medscape