Clinical review of 63 cases of sex cord stromal tumors.

PURPOSE OF INVESTIGATION: A retrospective analysis of 63 cases of sex cord stromal tumors treated in a 22-year period to evaluate the prognostic impact of different clinical parameters.

METHODS: Sixty-three cases of sex cord stromal tumors were studied. These neoplasms are characteristically detected at an early stage and may recur locally years after the initial diagnosis. The most frequent cell type was adult granulosa cell tumor (75%); a total of 37 patients (62%) had Stage IA lesions.

RESULTS: The cornerstone of treatment is surgery. Conservative surgical treatment was performed in 11 out of 47 cases (23%) of early stage tumor and in one of 13 patients affected by advanced neoplasm. Five of these 12 patients became pregnant after the treatment. Endometrial hyperplasia and uterine adenocarcinoma were diagnosed in 26.5% and 8.8% of the cases, respectively. Twenty-one patients (35%) received adjuvant therapy: 20 chemotherapy and one chemo-radiation treatment. Eight patients (13%) either progressed or recurred. All the recurrent patients but one had been treated with adjuvant chemotherapy (VAC and/or PVB). Overall survival by stage was 88.2% for Stage I and 30% for Stage III-IV.

CONCLUSION: Tumor stage is the most important clinical parameter of prognostic relevance. Tumor size and laterality significantly affected prognosis in terms of overall survival; survival rate did not seem to be affected either by the age of the patients or by the modality of surgical treatment.