Ovarian sex cord tumor with annular tubules. Clinicopathologic report of two benign and one malignant cases with long follow-ups

Abstract

The clinicopathologic features of three new cases of ovarian sex cord tumors with annular tubules are presented, thereby increasing to 23 the number of the published cases in the world literature. These three observations, along with another one which was previously published, were found in the files of the Institute of Pathology of the University of Lausanne from 1939 to 1978. Forty-seven granulosa cell tumors and eight Sertoli and/or Leydig cell tumors of the ovary were found during the same 40-year period. The patients were 48, 64 and 71 years of age. No sign of the Peutz-Jeghers syndrome was noticed in the three patients. All three tumors caused metrorrhagias as a cardinal sign. They were bulky, unilateral and were formed by solid tissue with cystic spaces. Histologically, the most characteristic pattern consisted of simple and complex tubular structures as described by Scully in 1970. Two patients, in which the mitotic indexes of the tumors were lower than 5 mitoses per 10 HPF, died without evidence of a recurrence 36 and 37 years after surgical ablation of the tumor. The third patient, whose neoplasm featured fewer well differentiated tubular structures than the two previous ones and had a mitotic index of over 70 mitoses per 10 HPF, died from massive abdominal recurrence after 5 years and 5 months.