Functioning ovarian tumors with peripheral steroid cell proliferation: a report of twenty-four cases

Twenty-four cases of an unusual type of ovarian tumor with functioning stroma in which bands of steroid-hormone-producing cells were located adjacent to the tumor are presented. The cells were of stromal lutein cell type in 14 cases, stromal Leydig cell type in two cases, and hilus cell type in eight cases. Of the tumors with peripheral lutein cells, 11 were monodermal teratomas, including eight examples of struma ovarii, two strumal carcinoids, and one trabecular carcinoid; the other three tumors in this group were a dermoid cyst, a dysgerminoma containing syncytiotrophoblast cells, and a metastatic carcinoid. The tumors with peripheral stromal Leydig cells were a struma ovarii and a strumal carcinoid. Three of the tumors with peripheral hilus cells were mucinous cystadenomas, four were cysts of probable rete origin, and the eight was a dermoid cyst. The patients' ages ranged from 13-82 (average 58) years. Eighty percent of the patients were postmenopausal; one was pregnant. Forty-two percent of the patients had androgenic manifestations, 29% had evidence of hyperestrinism, and one (4%) had evidence of progesterone excess. In 29% of the cases there was no clinical evidence of hormonal imbalance. Those tumors with peripheral stromal Leydig cells or hilus cells were accompanied by androgenic manifestations in over one-half the cases, and those tumors with peripheral lutein cells had a slightly higher association with estrogenic rather than androgenic changes. Human chorionic gonadotropin and its beta subunit were demonstrated by the immunoperoxidase technique in some neoplastic cells in 4 of the 17 cases in which it was performed. Ovarian tumors with peripheral steroid-hormone-producing cells should be distinguished from the typical ovarian tumor with functioning stroma, which contains lutein cells scattered throughout its stroma.