Endocrine and morphological study of a case of ovarian sex-cord tumor with annular tubules in a woman with Peutz-Jeghers syndrome

Abstract

A bilateral ovarian sex-cord tumor with annular tubules (SCTAT) was incidentally discovered in an amenorrheic patient with Peutz-Jeghers syndrome during conservative surgery in which a small non-capsulated mass was removed from each ovary. Ovulation was then induced over two consecutive cycles with urinary gonadotropins; the couple did not conceive because of a male infertility factor. Hysterectomy and bilateral oophorectomy were performed to prevent recurrence and avoid the possibility of a cervical malignant adenoma. Immunohistochemistry of the SCTAT showed positivity for estradiol and testosterone similar to that of Sertoli and granulosa cell tumors; progesterone was not detected in any cellular component of the neoplasia. Electron microscopy showed that the neoplasm consisted of numerous solid cords of cells surrounded by fibrillary layers of basal lamina, as well as central hyaline bodies. Two types of cells, clear and dark, were noted; clear cells were predominant and intermixed with scattered dark cells. No crystalloids or Charchot-Bottcher filaments were detectable in the tumors.