From the Department of Gynecologic Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas
Authors’ disclosures of potential conflicts of interest are found at the end of this article.
Address reprint requests to Judith K. Wolf, MD, Department of Gynecologic Oncology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Unit 1362, Houston, TX 77030; e-mail: jwolf{at}mdanderson.org
Overview: Sex cord-stromal tumors originate from specialized gonadal stromal cells and their precursors. Granulosa and Sertoli cells arise from sex cord cells, whereas theca cells, Leydig cells, lipid cells, and fibroblasts arise from stromal cells and their pluripotential mesenchymal precursors. These tumors can occur as a single histologic type or a combination of types, and together they account for 7% of all ovarian malignancies. Because tumor cells from sex cord-stromal malignancies can be involved in steroid hormone production, physical manifestations of excess estrogen or androgen production are not infrequent at the time of diagnosis. Although the majority of these tumors are clinically indolent and have a good long-term prognosis, many occur in adolescents and women of reproductive age. Therefore, individualized treatment after appropriate guidelines is the key to successful outcomes, including the maintenance of fertility.
Although the majority of ovarian cancers are epithelial in origin, 10% are nonepithelial. Most of these are sex cord-stromal tumors and germ cell tumors. Surgery, chemotherapy, radiation therapy, and hormone therapy are components of treatment for these rare ovarian tumors, but specific treatment recommendations are based on many factors, including histologic type, extent of disease, and patient age. Conservative surgery to maintain reproductive potential is an important consideration in many patients because some sex cord-stromal tumors occur in adolescents and young women. Appropriate surgical staging and assessment, however, are necessary for determining the extent of surgery and the need for postoperative chemotherapy. With the advent of modern surgical and postsurgical techniques, response rates and survival have improved dramatically and are excellent for most types of nonepithelial ovarian tumors.
Treatment of nonepithelial ovarian cancers is determined by many factors, including patient age, parity, desire for future fertility, extent of disease, comorbid conditions, and karyotype. Often the gynecologist or gynecologic oncologist has a patient with an adnexal mass, the precise histologic classification of which is difficult to determine, even with the pathologic evaluation of frozen tissue sections. The surgeon must then follow general guidelines for nonepithelial ovarian tumors during the initial operative management and re-evaluate the need for adjuvant or additional therapy on the basis of the final pathologic results. With close attention to all details, including histologic type, patient characteristics, and extent of disease, the need for re-exploration and more extensive surgery can be minimized.
