Sex Cord Stromal Tumors

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Sex cord-stromal tumors consist of a pathologically diverse group of ovarian tumors originating from the sex cords and stroma of the ovary. Their exact histogenesis remains unclear. Collectively, they account for 5% to 8% of all ovarian tumors and can occur in women of any age group, but they are most commonly diagnosed in women in the fifth decade of life. Sex cord-stromal tumors are classified into granulosa cell tumors (GCTs), Sertoli-Leydig tumors, sex cord-stromal tumors with annular tubules, and gynandroblastomas39 (Table 11-3).

Sex cord-stromal tumors display intermediate malignant behavior in that they can vary from benign to malignant, depending on cell type and level of differentiation. Fortunately, most are diagnosed at an early stage owing to their hormonal activity, which is responsible for the unique presenting symptoms of these tumors. GCTs produce estrogen, whereas Sertoli-Leydig tumors secrete testosterone. Other

Table 11-3. World Health Organization Classification of Sex Cord-Stromal Tumors Sex Cord-Stromal Tumor Types Percentage

2.1 Granulosa-stromal cell tumors: Granulosa cell tumors thecoma-fibroma 70

2.2 Sertoli-stromal cell tumors, androblastomas: Well-differentiated, Sertoli-Leydig <0.2 cell tumor of intermediate differentiation, Sertoli-Leydig cell tumor poorly differentiated (sarcomatoid), retiform

2.3 Sex cord tumor with annular tubules

2.4 Gynandroblastoma <1

2.5 Unclassified <1

2.6 Steroid (lipid) cell tumors: Stromal luteoma, Leydig cell tumor, unclassified <1

From WHO Histological Typing of Ovarian Tumours. Copyright 1999.

sex cord-stromal tumors may also secrete a combination of hormones. In estrogen-secreting tumors, breast enlargement and vaginal bleeding are the most common symptoms. Women of reproductive age may present with menstrual irregularities. The postmenopausal female typically presents with postmenopausal bleeding and breast tenderness. In androgen-secreting tumors, hirsutism, deepening of the voice, male-pattern baldness, breast shrinkage, clitoromegaly, and acne may occur.

Additional nonspecific complaints result from tumor mass, including increased abdominal girth, bloating, and palpable mass on physical examination. Urinary complaints and constipation accompany tumor compression of the bladder and/or rectum.

Overall prognosis is good for these tumors, since most sex cord-stromal tumors are diagnosed at an early stage, have indolent growth, and recur later than their epithelial counterparts. Although some sex cord-stromal tumors are purely benign (e.g., fibromas and thecomas), others have the potential to behave more aggressively. Fortunately, over 90% of sex cord-stromal tumors are diagnosed at stage I. Unequivocally, surgery is the initial step in management for both diagnosis and treatment.

Treatment for sex cord-stromal tumors is controversial because these tumors are relatively rare. The general consensus for postmenopausal women includes total abdominal hysterectomy and bilateral salpingo-oophorectomy with complete surgical staging. Staging should include pelvic washings, peritoneal biopsies, omen-tectomy, and pelvic and para-aortic lymph node sampling. In a woman who desires future fertility, it is acceptable to leave the remaining ovary and uterus as long as (1) the tumor is confined to one ovary, (2) the contralateral ovary appears normal, and (3) a thorough inspection of the abdomen and pelvis reveals no extraovarian disease.

Unfortunately, for women diagnosed in advanced stages, there are no available randomized controlled trials to guide management, and prospective trials are limited as a consequence of their relative rarity. Most of the available literature is derived from retrospective institutional experiences. In general, for patients with advanced disease, recurrence, or poorly differentiated tumors, adjuvant chemotherapy with bleomycin/etoposide/cisplatin (BEP) or a platinum/taxane regimen is recommended.

Although there is no consensus regarding surveillance of women with sex cord-stromal tumors, given the potential for late recurrence these women require lifetime surveillance. In our current practice, we see these women every 3 to 4 months for the first 2 years, every 6 months in years 3 to 5, and annually thereafter. At each visit, a pelvic examination is performed, and tumor-specific markers are obtained. Some providers obtain periodic CT scans; others acquire only images if there is concern for recurrence.

Because sex cord-stromal tumors are so diverse, each is discussed separately with regard to epidemiology, histology, diagnosis, and treatment recommendations.

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