Sex cord-stromal tumors of the ovary: Tumors with granulosa and Sertoli-Leydig elements
- David M Gershenson, MD
David M Gershenson, MD
- J Taylor Wharton MD Distinguished Chair in Gynecologic Oncology
- Professor of Gynecologic Oncology
- The University of Texas MD Anderson Cancer Center
- Section Editors
- Barbara Goff, MD
Barbara Goff, MD
- Section Editor — Gynecologic Oncology
- Professor of Gynecologic Oncology
- University of Washington
- Rochelle L Garcia, MD
Rochelle L Garcia, MD
- Section Editor — Obstetric and Gynecologic Pathology
- Professor of Pathology
- Adjunct Professor of Obstetrics & Gynecology
- University of Washington Medical Center
- Don S Dizon, MD, FACP
Don S Dizon, MD, FACP
- Section Editor – Gynecologic Oncology
- Clinical Co-Director, Gynecologic Oncology
- Founder and Director, The Oncology Sexual Health Clinic
- Massachusetts General Hospital Cancer Center
- Associate Professor of Medicine
- Harvard Medical School
Ovarian sex cord-stromal tumors are a heterogeneous group of benign or malignant neoplasms that develop from the dividing cells that normally produce cells that support and surround the oocytes, including the cells that produce ovarian hormones (the nongerm cell and nonepithelial components of the gonads) (figure 1) . Ovarian sex cord-stromal tumors are rare, comprising only 1.2 percent of all primary ovarian cancers .
In contrast with epithelial ovarian cancer, most patients with malignant sex cord-stromal tumors are diagnosed with early-stage disease; the neoplasms are generally considered to be low-grade malignant neoplasms.
Diagnosis of sex-cord stromal tumors is made by histology at time of surgical excision. Staging for malignant tumors is the same as for other primary ovarian carcinomas (table 1).
Ovarian sex cord-stromal tumors with both granulosa and Sertoli-Leydig cell elements (sex cord tumor with annular tubules and other tumors) are reviewed here. An overview of sex cord-stromal tumors and discussions about other types of sex cord-stromal tumors of the ovary (Sertoli-stromal cell tumors and tumors with granulosa and Sertoli-Leydig elements), as well as epithelial ovarian cancer, can be found separately. (See "Overview of sex cord-stromal tumors of the ovary" and "Sex cord-stromal tumors of the ovary: Granulosa-stromal cell tumors" and "Sex cord-stromal tumors of the ovary: Sertoli-stromal cell tumors" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Histopathology" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Clinical features and diagnosis".)
SEX CORD TUMOR WITH ANNULAR TUBULES
These are unique types of sex cord-stromal tumors that are characterized by simple and complex annular (ring-shaped) tubules; they may be benign or malignant. They are thought to represent an intermediate between Sertoli cell and granulosa cell tumors. In one institutional series, sex cord tumors with annular tubules (SCTAT) accounted for 6 percent of all ovarian sex cord-stromal tumors .
- Young RH. Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems. Mod Pathol 2005; 18 Suppl 2:S81.
- Quirk JT, Natarajan N. Ovarian cancer incidence in the United States, 1992-1999. Gynecol Oncol 2005; 97:519.
- Shen K, Wu PC, Lang JH, et al. Ovarian sex cord tumor with annular tubules: a report of six cases. Gynecol Oncol 1993; 48:180.
- Westerman AM, Wilson JH. Peutz-Jeghers syndrome: risks of a hereditary condition. Scand J Gastroenterol Suppl 1999; 230:64.
- Young RH, Welch WR, Dickersin GR, Scully RE. Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer 1982; 50:1384.
- Puls LE, Hamous J, Morrow MS, et al. Recurrent ovarian sex cord tumor with annular tubules: tumor marker and chemotherapy experience. Gynecol Oncol 1994; 54:396.
- Gustafson ML, Lee MM, Scully RE, et al. Müllerian inhibiting substance as a marker for ovarian sex-cord tumor. N Engl J Med 1992; 326:466.
- Crain JL. Ovarian sex cord tumor with annular tubules: steroid profile. Obstet Gynecol 1986; 68:75S.
- Hart WR, Kumar N, Crissman JD. Ovarian neoplasms resembling sex cord tumors with annular tubules. Cancer 1980; 45:2352.
- Lele SM, Sawh RN, Zaharopoulos P, et al. Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: a case report. Mod Pathol 2000; 13:466.
- Rodu B, Martinez MG Jr. Peutz-Jeghers syndrome and cancer. Oral Surg Oral Med Oral Pathol 1984; 58:584.
- Barker D, Sharma R, McIndoe A, et al. An unusual case of sex cord tumor with annular tubules with malignant transformation in a patient with Peutz-Jeghers syndrome. Int J Gynecol Pathol 2010; 29:27.
- Tsuruchi N, Tsukamoto N, Kaku T, et al. Adenoma malignum of the uterine cervix detected by imaging methods in a patient with Peutz-Jeghers syndrome. Gynecol Oncol 1994; 54:232.
- Srivatsa PJ, Keeney GL, Podratz KC. Disseminated cervical adenoma malignum and bilateral ovarian sex cord tumors with annular tubules associated with Peutz-Jeghers syndrome. Gynecol Oncol 1994; 53:256.
- Ahn GH, Chi JG, Lee SK. Ovarian sex cord tumor with annular tubules. Cancer 1986; 57:1066.
- Fukunaga M, Endo Y, Ushigome S. Gynandroblastoma of the ovary: a case report with an immunohistochemical and ultrastructural study. Virchows Arch 1997; 430:77.
- Martin-Jimenez A, Condom-Munró E, Valls-Porcel M, et al. [Gynandroblastoma of the ovary. Review of the literature]. J Gynecol Obstet Biol Reprod (Paris) 1994; 23:391.
- Novak ER. Gynandroblastoma of the ovary: review of 8 cases from the ovarian tumor registry. Obstet Gynecol 1967; 30:709.