Sex cord stromal tumors-unusual presentations

The sex cord stromal tumors (SCST) account for approximately 7% of all malignant ovarian tumors. They are endocrinologically active tumors of which granulosa cell tumors are the commonest. We describe an analysis of all cases of SCST managed, in our department over the last five years and discuss two cases of unusual presentation of these tumors.

Ten cases of SCST which were managed in the Department of Gynecology and Obstetrics at University College of Medical Sciences (UCMS) & associated Guru Teg Bahadur hospital, Delhi from Jan. 01 to Dec. 05 were analyzed.

Of the total 312 ovarian tumors which were operated in these 5 years, 73 (23.3%) were malignant, 10 cases (13.6%) out of the malignant ovarian tumors were SCST. The age of presentation varied from 4 to 70 years. Four women presented with lump abdomen as their chief complaint, 2 had postmenopausal bleeding, 2 presented with irregular bleeding per-vaginum, 1 woman had secondary amenorrhea and one young girl who was only 4½ years old developed precocious puberty. The majority of cases (70%) were granulosa cell tumors, 10% were fibromas and 20% thecomas. Maximum patients 9 (90%) were stage I at the time of surgery and only 1 case was stage IV with tumor infiltrating into omentum and gut.

SCST tumors are rare endocrine active tumors that are essentially unilateral, benign or with a low malignant potential. Though most patients present with one or a combination of symptoms ranging from abnormal vaginal bleeding, abdominal distention and abdominal pain, the signs of Paraneoplastic syndrome (PNS) may be the first indication of the presence of an ovarian malignancy and can even facilitate its diagnosis.