Sex cord-stromal tumors of the ovary: Sertoli-stromal cell tumors
- 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
- Department Chair, Gynecologic Oncology
- University of Washington Medical Center
- 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
- Head of Women's Cancers, Lifespan Cancer Institute
- Director of Medical Oncology, Rhode Island Hospital
- Associate Professor of Medicine, Warren Alpert Medical School of Brown University
- Deputy Editors
- Sandy J Falk, MD, FACOG
Sandy J Falk, MD, FACOG
- Director, Editorial Relations — UpToDate
- Deputy Editor — Obstetrics, Gynecology and Women's Health
- Instructor of Obstetrics, Gynecology and Reproductive Biology, Part-time
- Harvard Medical School
- Sadhna R Vora, MD
Sadhna R Vora, MD
- Deputy Editor — Oncology
- Instructor in Medicine
- Harvard Medical School
Ovarian sex cord-stromal tumors are a heterogeneous group of benign or malignant neoplasms that develop from the stem cells that would typically furnish cells surrounding 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 tumors are generally considered to be low-grade malignant neoplasms.
Sertoli-Leydig cell tumors constitute less than 0.5 percent of ovarian neoplasms . They may behave in a benign or malignant fashion, which correlates with the degree of differentiation in an individual case. Approximately 75 percent occur in women under the age of 40 years (mean age at diagnosis is 25), but they occur in all age groups. The neoplasms are characterized by the presence of testicular structures that produce androgens. This can result in virilization, although not all of these neoplasms are functionally active.
Ovarian sex cord-stromal tumors of the Sertoli-stromal cell type are reviewed here. This category includes Sertoli-Leydig cell tumors (androblastomas) as well as pure Sertoli cell or Leydig cell tumors. An overview of sex cord-stromal tumors and 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, are discussed 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: Tumors with granulosa and Sertoli-Leydig elements" 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".)
Two gene mutations associated with Sertoli-Leydig cell tumors have been reported, and both may be clinically relevant. FOXL2 is a somatic missense point mutation that was initially reported to be present in granulosa cell tumors of the ovary . Subsequently, the same group analyzed this marker in a larger series of sex cord-stromal ovarian tumors . The authors found that FOXL2 is a relatively sensitive and highly specific marker for this tumor type. FOXL2 immunostaining was present in almost all sex cord-stromal tumors with a FOXL2 mutation and in a majority of tumors without a mutation. Importantly, only 50 percent of Sertoli-Leydig cell tumors expressed FOXL2. However, together with alpha-inhibin and calretinin, FOXL2 forms an immunomarker panel that will aid in the diagnosis of a sex cord-stromal ovarian tumor.
Subscribers log in hereLiterature review current through: Nov 2017. | This topic last updated: Feb 07, 2017.References
- 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.
- DiSaia PJ, Creasman WT. Germ cell, stromal and other ovarian tumors. In: Clinical Gynecologic Oncology, Mosby-Yearbook, 1997. p.351.
- Shah SP, Köbel M, Senz J, et al. Mutation of FOXL2 in granulosa-cell tumors of the ovary. N Engl J Med 2009; 360:2719.
- Al-Agha OM, Huwait HF, Chow C, et al. FOXL2 is a sensitive and specific marker for sex cord-stromal tumors of the ovary. Am J Surg Pathol 2011; 35:484.
- Schultz KA, Pacheco MC, Yang J, et al. Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol 2011; 122:246.
- Heravi-Moussavi A, Anglesio MS, Cheng SW, et al. Recurrent somatic DICER1 mutations in nonepithelial ovarian cancers. N Engl J Med 2012; 366:234.
- Young R, Clement PB, Scully RE. The ovary. In: Surgical Pathology, Sternberg SS (Ed), Raven Press, New York 1989. p.1687.
- Mooney EE, Nogales FF, Tavassoli FA. Hepatocytic differentiation in retiform Sertoli-Leydig cell tumors: distinguishing a heterologous element from Leydig cells. Hum Pathol 1999; 30:611.
- Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol 1985; 9:543.
- Horny HP, Braumann W, Weiss E, et al. Virilizing stromal Leydig cell tumor (Leydig cell-containing thecoma) of the ovary in pregnancy. A case report with extensive immunohistochemical investigation of the tumor cells. Gen Diagn Pathol 1995; 141:57.
- Gui T, Cao D, Shen K, et al. A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors. Gynecol Oncol 2012; 127:384.
- Oliva E, Alvarez T, Young RH. Sertoli cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol 2005; 29:143.
- Roth LM, Sternberg WH. Ovarian stromal tumors containing Leydig cells. II. Pure Leydig cell tumor, non-hilar type. Cancer 1973; 32:952.
- Cohen I, Shapira M, Cuperman S, et al. Direct in-vivo detection of atypical hormonal expression of a Sertoli-Leydig cell tumour following stimulation with human chorionic gonadotrophin. Clin Endocrinol (Oxf) 1993; 39:491.
- Ohashi M, Hasegawa Y, Haji M, et al. Production of immunoreactive inhibin by a virilizing ovarian tumour (Sertoli-Leydig tumour). Clin Endocrinol (Oxf) 1990; 33:613.
- Gagnon S, Têtu B, Silva EG, McCaughey WT. Frequency of alpha-fetoprotein production by Sertoli-Leydig cell tumors of the ovary: an immunohistochemical study of eight cases. Mod Pathol 1989; 2:63.
- Gershenson DM. Management of early ovarian cancer: germ cell and sex cord-stromal tumors. Gynecol Oncol 1994; 55:S62.
- Zhang M, Cheung MK, Shin JY, et al. Prognostic factors responsible for survival in sex cord stromal tumors of the ovary--an analysis of 376 women. Gynecol Oncol 2007; 104:396.
- Brown J, Sood AK, Deavers MT, et al. Patterns of metastasis in sex cord-stromal tumors of the ovary: can routine staging lymphadenectomy be omitted? Gynecol Oncol 2009; 113:86.
- National Comprehensive Cancer Network (NCCN). NCCN Clinical practice guidelines in oncology. http://www.nccn.org/professionals/physician_gls/f_guidelines.asp (Accessed on February 27, 2016).
- Schneider DT, Calaminus G, Wessalowski R, et al. Ovarian sex cord-stromal tumors in children and adolescents. J Clin Oncol 2003; 21:2357.
- Li B, Wu LY, Zhang WH, et al. [Clinical analysis of 11 cases of ovarian Setoli-Leydig cell tumor]. Zhonghua Fu Chan Ke Za Zhi 2004; 39:334.
- Sigismondi C, Gadducci A, Lorusso D, et al. Ovarian Sertoli-Leydig cell tumors. a retrospective MITO study. Gynecol Oncol 2012; 125:673.
- Homesley HD, Bundy BN, Hurteau JA, Roth LM. Bleomycin, etoposide, and cisplatin combination therapy of ovarian granulosa cell tumors and other stromal malignancies: A Gynecologic Oncology Group study. Gynecol Oncol 1999; 72:131.
- Gershenson DM, Morris M, Burke TW, et al. Treatment of poor-prognosis sex cord-stromal tumors of the ovary with the combination of bleomycin, etoposide, and cisplatin. Obstet Gynecol 1996; 87:527.
- Gershenson DM, Copeland LJ, Kavanagh JJ, et al. Treatment of metastatic stromal tumors of the ovary with cisplatin, doxorubicin, and cyclophosphamide. Obstet Gynecol 1987; 70:765.
- Tomlinson MW, Treadwell MC, Deppe G. Platinum based chemotherapy to treat recurrent Sertoli-Leydig cell ovarian carcinoma during pregnancy. Eur J Gynaecol Oncol 1997; 18:44.
- Fujimoto A, Saitou M, Ishihara O, et al. [A case of ovarian malignant Sertoli-Leidig cell tumor treated with CBDCA, etoposide and epirubicin chemotherapy]. Gan To Kagaku Ryoho 1995; 22:1843.
- van der Meer J, de Vries EG, Vriesendorp R, et al. Hemolytic uremic syndrome in a patient on cis-platinum, vinblastine and bleomycin. J Cancer Res Clin Oncol 1985; 110:119.
- Brown J, Shvartsman HS, Deavers MT, et al. The activity of taxanes in the treatment of sex cord-stromal ovarian tumors. J Clin Oncol 2004; 22:3517.