Sertolileydig Cell Tumors Treatment Issues

This classification of tumors includes tumors comprised of Sertoli cells only, as well as tumors containing both Sertoli and Leydig cells. Tumors composed of Sertoli cells only are uniformly stage I, and only one death has been reported [64]. Sertoli-Leydig cell tumors, also called arrhenoblastomas, are rare, accounting for less than 0.2% of all ovarian tumors, and usually present in adolescents and young adults. Since stage is the most important predictor of outcome, these patients should be accurately staged (Table 13.5). Over 95% of these tumors are confined to one ovary at the time of diagnosis; therefore, a normal-appearing uterus and contralateral ovary can usually be preserved. Patients with disease greater than IB, with poorly differentiated tumors, or with heterolo-gous elements present should be treated with BEP [65] or paclitaxel and carboplatin. Patients can be followed with physical examinations and with serum aFP, inhibin, and testosterone levels. Of the 18% of patients who recur, two-thirds do so within the 1st year after

Figure 13.3

Five-year survival rates of females with ovarian malignancies, United States SEER [1]

Figure 13.4

Five-year survival rates of females with ovarian malignancies as a function of era, United States SEER [1]

diagnosis. Additional platinum-based chemotherapy is the mainstay of treatment for recurrent disease.

A subgroup of the sex-cord tumor grouping is the ovarian sex-cord tumor with annular tubules. The biologic behavior of this lesion is thought to be intermediate between granulosa cell tumors and Sertoli cell tumors, and is associated with Peutz-Jeghers Syndrome [30, 31]. Surgical treatment recommendations are similar to those for other sex-cord stromal tumors, but patients should be carefully screened for adenoma malignum of the cervix, as 15% of patients harbor an occult lesion. For such patients, hysterectomy must be strongly considered [66].

Steroid cell tumors not otherwise specified are a distinct category of steroid cell tumor that can be malignant and aggressive; when diagnosed intraopera-tively, these patients should be staged and aggressively cyto-reduced [67, 68]. Lipid cell tumors with pleomor-phism, increased mitotic count, large size, or advanced stage should receive additional post-operative platinum-based therapy [68].

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