Sex cord tumor with annular tubules a distinctive ovarian tumor of the Peutz-Jeghers syndrome

Abstract

After the observation that 3 rare, distinctive-appearing ovarian sex cord tumors in our files occurred in women with the Peutz-Jeghers syndrome, a review of the reported cases of ovarian neoplasms associated with that disorder was undertaken. Examination of the slides or photomicrographs of 12 of the 13 tumors in the literature disclosed 3 additional cases of this unusual neoplasm. It is characterized by the formation of simple and complex annular tubules, and multicentricity and calcification are common features. The tumor, which is thought to arise from granulosa cells, but to grow in a pattern more characteristic of Sertoli cells, can be appropriately designated “sex cord tumor with annular tubules.” Endometrial hyperplasia may be associated and the discovery of this type of tumor may be the first clue to the diagnosis of the Peutz-Jeghers syndrome. Clinicians should be aware of the possibility of genital tract abnormalities in women with this disorder, and pathologists should examine their ovarian tissue, whenever available, with great care.