UNCLASSIFIED SEX CORD-STROMAL TUMORS OF THE OVARY - A REPORT OF 8 CASES

Objective. - To evaluate the histologic features and biologic behavior of unclassified sex cord-stromal tumors. Patients. - The eight patien ts' ages at presentation ranged from 14 to 83 years. Presenting sympto ms and physical findings included abdominal pain, abnormal uterine ble eding, ascites, and abdominal and pelvic masses. One patient also had bilateral sex cord tumors with annual tubules and probable Peutz-Jeghe rs syndrome. Results. - The tumors ranged from 4 to 27 cm in diameter and were described as partially encapsulated, solid, and cystic. Histo logically, the tumors were composed of diffuse proliferations of sex c ord cells, with cords, tubules, and follicle-like structures. The stro mal cells were spindle-shaped, with scanty cytoplasm. The neoplasms we re vimentin-positive and, sometimes, cytokeratin CAM 5.2 - and AE1/3-p ositive and epithelial membrane antigen-negative. Six patients were di sease-free from 2 months to 6 years after operation. One patient was l ost to follow-up. The patient with probable Peutz-Jeghers syndrome had a tumor with unusual morphology and died of the neoplasm 4 years afte r the diagnosis. Three of 32 other cases with clinical follow-up menti oned in the pathology literature have been associated with a malignant behavior. Conclusion. - The biologic behavior of unclassified sex cor d-stromal tumors resembles that of Sertoli-Leydig cell tumors of inter mediate differentiation rather than poorly differentiated tumors, whic h might have been expected in view of the lack of specific differentia tion. This finding is important with regard to postoperative managemen t.