Sex Cord Tumor with Annular Tubules

Sex cord tumors with annular tubules (SCTATs) were initially grouped with SLCTs, and some have proposed grouping them with GCTs. Correspondingly, these tumors possess characteristics of both, but they are recognized as a unique entity. Distinctive features include an association with Peutz-Jeghers syndrome in one third of cases, the potential to produce progesterone, and a higher penchant for lymph node metastasis than other sex cord-stromal tumors.84

Presentation

The presentation of SCTATs differs based on their association with Peutz-Jeghers syndrome (PJS). In those with Peutz-Jeghers syndrome, tumors are small, bilateral, calcified, and asymptomatic. Given their small size, they are typically not palpable on examination. In contrast, in non-Peutz-Jeghers-associated cases, the population is younger (20s), and tumors are larger, unilateral, and symptomatic.85 They are hor-

monally active, often secreting estrogen and/or progesterone, which in a younger target population often presents as menstrual cycle disruptions or dysfunctional uterine bleeding.

Histology

SCTATs are thought of as a combination of granulosa cell and Sertoli-Leydig tumors. The annular (circular) tubules correspond to Sertoli cells, but contain false lumens. They also contain histologic elements consistent with GCTs, and they secrete estradiol. As in GCTs, estradiol is not a useful tumor marker, but inhibin may be useful for surveillance purposes. Progesterone levels also prove useful in the case of progesterone-producing tumors.

Treatment

The recommended treatment for SCTATs is surgical resection. In patients with Peutz-Jeghers syndrome, these tumors are uniformly benign; therefore, treatment consists of unilateral oophorectomy. An interesting association exists between Peutz-Jeghers-associated tumors and malignant adenoma of the cervix, which has been reported in up to 15% of cases.85 Therefore, these patients require close surveillance with a low threshold for excisional biopsy, since cytology and colposcopy are often nondiagnostic. In non-Peutz-Jeghers-associated cases, tumors behave more aggressively. The risk of extraovarian spread and metastasis is roughly 20% at the time of initial surgery.85,86 In a study of six patients with SCTATs, lymph node metastases occurred in two patients 7.5 and 10 years after surgery, but all patients were alive at a mean of 7.8 years.87 Another review examined four patients, one of whom had metastasis to a supraclavicular lymph node, the liver, and the retroperitoneum.88 Treatment still consists of surgery with complete staging. Adjuvant chemotherapy may offer some additional survival benefit. Typical regimens include BEP or a plati-num/taxane combination, but experience is limited.

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