Sex Cord Stromal Tumors of the Ovary

Ovarian sex cord and stromal tumors represent a heterogeneous group of rare tumors arising from the ovarian stroma and cells that surround the oocytes. Their rarity precludes any definite recommendations regarding their management. Surgery is the cornerstone of therapy and is required for definitive diagnosis. The staging system is the same as that for epithelial ovarian cancer. Most malignant ovarian sex cord-stromal tumors, however, tend to present at an earlier age and stage and to follow an indolent course and recur late, and can thus be managed more conservatively. In their review of 83 women with sex cord-stromal tumors of the ovary, Chan and colleagues108 reported that nearly 50% of their patients were less than 50 years of age and over 70% presented with early-stage disease. They were able to confirm that, similar to previous reports, tumor size (less than 10 cm) and absence of residual disease improved prognosis with a nearly 8% decrease in risk of death for every 1 cm decrease in size and an improvement of 66 months in overall survival for patients left with no residual disease108 (Fig. 7-27). Although the investigators did not report a significant difference in overall survival between unstaged and staged patients, the recurrence rate was significantly higher for patients who were not staged (64% versus 28%). Since most sex cord-stromal tumors tend to secrete sex steroids including estrogen, up to 55% of cases are associated with concurrent endometrial hyperplasia and up to 10% with a synchronous uterine adenocarcinoma.109 In postmenopausal women and women having completed their childbearing, a total abdominal hysterectomy and bilateral sal-pingo-oophorectomy is recommended; if the patient desires to conserve fertility, a unilateral salpingo-oophorectomy is acceptable treatment for early-stage disease as long as a preoperative endometrial biopsy is performed to exclude the possibility of a synchronous endometrial carcinoma. Treatment for recurrent or metastatic disease is primarily surgical, with reports of long-term disease control with complete resection of localized recurrences110 (Fig. 7-28), since these tumors tend to be relatively chemotherapy- and radiation-resistant.111

Figure 7-27. Kaplan-Meier survival analysis based on the residual tumor status for 83 patients with sex cord stromal tumors of the ovary. (From Chan JK, Zhang M, Kaleb V, et al: Prognostic factors responsible for survival in sex cord stromal tumors of the ovary—a multivariate analysis. Gynecol Oncol 96:204-209, 2005, Figure 4.)

Survival (months)

Residual tumor

No residual tumor

Figure 7-27. Kaplan-Meier survival analysis based on the residual tumor status for 83 patients with sex cord stromal tumors of the ovary. (From Chan JK, Zhang M, Kaleb V, et al: Prognostic factors responsible for survival in sex cord stromal tumors of the ovary—a multivariate analysis. Gynecol Oncol 96:204-209, 2005, Figure 4.)

Figure 7-28. Kaplan-Meier survival analysis based on the residual tumor status of 65 patients with granulosa cell tumor of the ovary. (From Sehouli J, Drescher FS, Mustea A, et al: Granulosa cell tumor of the ovary: 10 years follow-up data of 65 patients. Anticancer Res 24:1223-1239, 2004, Figure 7.)

20 40 60 80 100 120 140

Time in months

20 40 60 80 100 120 140

Time in months

Residual disease Complete removal

Figure 7-28. Kaplan-Meier survival analysis based on the residual tumor status of 65 patients with granulosa cell tumor of the ovary. (From Sehouli J, Drescher FS, Mustea A, et al: Granulosa cell tumor of the ovary: 10 years follow-up data of 65 patients. Anticancer Res 24:1223-1239, 2004, Figure 7.)

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