Uterine tumor resembling ovarian sex cord tumors treated by resectoscopic surgery.

Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are rare neoplasms of uncertain malignancy, affecting either pre or menopausal women. Only 48 cases of UTROSCTs have been reported and in most patients the diagnosis was made incidentally, after a hysterectomy as a result of the assumption of a leiomyoma. Although no death from progressive disease was reported, locoregional spread or abdominal relapse was detected in some patients. Up until now, a treatment sparing the uterus was reported in only 2 patients with UTROSCTs. Both patients conceived after surgery and were free from recurrent disease after a mean follow-up of 16.5 months. We report a third case of a young patient affected by pure UTROSCTs and treated conservatively. A 29-year-old woman underwent resectoscopic hysteroscopy because of the assumption of a grade-1 submucous myoma measuring about 5 cm. To accomplish the removal of the endometrial mass, a 2-step hysteroscopic resection was required. Histology showed epithelioid cells arranged in nests, cords, tubules, and trabeculae infiltrating the myometrium; no sarcoma cell differentiation was detected. Immunohistochemistry resulted in positivity to CD99, inhibin, calretinin, cytokeratin, and estrogen receptor, whereas a negative staining to epithelial membrane antigen, actin, desmin, and human melanoma black 45 was obtained. Based on histologic pattern and immunohistochemical phenotype, a diagnosis of pure UTROSCTs was made; the staging of disease revealed neither locoregional spread nor distant metastases. Two months after surgery, reassessment by hysteroscopy and endometrial biopsy revealed no residual endometrial disease. The patient was free from disease 13 months after surgery. Because of the uncertain malignant potential of UTROSCTs and based on the poor data available from current literature, a fertility-sparing surgery in young patients appears safe. However, a close long-term follow-up is required and a quick completion of a patient's childbearing desire seems advisable.