Sertoli-Leydig cell tumors (SLCTs) are rare, accounting for 0.2% of all ovarian tumors. Unlike granulosa-stromal cell tumors, they occur principally in women in the reproductive years with 75% occurring before age 40 and occurring less commonly during menopause or childhood. Similar to theca cells, SLCTs are usually hormonally active and secrete testosterone, thus resulting in androgen excess and potential virilization.
The cause of SLCTs remains largely unknown because they originate from the ovarian stromal sex cords but resemble the Sertoli and Leydig cells found in testes. Some hypothesize that they originate from residual undifferentiated sex cord tissue, from mesenchymal, coelomic, or mesonephric cells. More recently, developments point to lack of estrogen, which induces transformation of ovarian follicles into structures resembling seminiferous tubules and Sertoli cells found in the testis.81 These, in turn, produce MIS and secrete testosterone.
The testosterone production associated with SLCTs may cause menstrual disruption, most notably amenorrhea, as well as hirsutism and virilization. Aside from secretion of testosterone, pure Sertoli cell tumors may secrete estrogen or renin. Thus, some patients present with dysfunctional uterine bleeding or hypertension and hypokalemia. Pure Leydig cell tumors secrete only testosterone. However, most tumors are combination SLCTs, and 70% to 85% manifest as virilizing symptoms such as clitoro-megaly secondary to androgen secretion (Fig. 11-5).
In any women presenting with virilization, it is necessary to consider other etiologies including Cushing's syndrome, adrenal tumors, pituitary dysfunction, adrenal hyper-plasia, or drug-induced hyperandrogenism. On physical examination, an adnexal mass may be detectable, in conjunction with symptoms associated with androgen excess. As in other ovarian tumors, ultrasound is used for diagnosis. Imaging typically reveals a well-circumscribed unilateral solid mass, or it may reveal a heterogeneously enlarged multicystic ovary (Fig. 11-6). Diagnosis relies on a combination of imaging and laboratory values, but it ultimately rests on surgery.
Grossly, over 95% of SLCTs are unilateral and mostly solid, but some have cystic components (Fig. 11-7). They are tan, gray, or white, and their size correlates with the degree of differentiation and prognosis. Thus, larger tumors tend to be poorly differentiated and more aggressive. Well-differentiated tumors are usually 3 to 4 cm and contain retiform (arranged like a net) tissue lending a spongy texture. Poorly differentiated tumors are more likely to contain areas of hemorrhagic necrosis. The hallmark of these tumors is a tubular pattern surrounded by fibrous tissue.
Histologically, SLCTs are classified into five different types: well-differentiated, intermediate differentiation, poorly differentiated, retiform, and mixed with heter-ologous elements. Heterologous elements include other types of tissue, for example gastrointestinal or cartilaginous. Retiform components may contain hepatocytic differentiation, which results in AFP production. Histology generally determines prognosis.
Definitive treatment for SLCTs is surgery with complete surgical staging. Over 90% of tumors are discovered in stage I, which lends itself to a good prognosis. Five-year survival rate is favorable, and ranges from 70% to 90%. In advanced stages, prognosis is poor and mortality rate approaches 100%. Young and Scully82 reviewed 207 cases of Sertoli-Leydig tumors and found that none of the well-differentiated tumors, 11% of intermediately differentiated tumors, 59% of poorly differentiated tumors, and 19% of the tumors with heterologous elements behaved malignantly. Thus, differentiation plays a large part in prognosis.
In the case of advanced disease or poorly differentiated SLCTs, adjuvant chemotherapy may provide benefit. BEP and PVB regimens have shown some response.66 About 33% of these tumors recur, and accordingly, poorly differentiated tumors are more likely to do so.83 Unlike GCTs, SLCTs tend to recur early—within 5 years—and sites of recurrence include the abdomen and pelvis.
Postoperatively, testosterone levels decline rapidly and may serve as a useful tumor marker. However, virilization in those with SLCTs may never resolve completely. As in GCTs, patients with SLCTs require lifetime surveillance. Imaging is generally utilized only when there is concern for recurrence by testosterone levels, patient symptoms, or physical examination findings.
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