Sex Cord Tumor with Annular Tubules Associated with Turner's Syndrome: Report of a case

ABSTRACT

An ovarian sex cord tumor with annular tubules(SCTAT) in an 18-year-old woman who had secondary amenorrhea but not Peutz-Jeghers syndrome was studied by light and electron microscopy. Her clinical features were consistent with those of Turner's syndrome, i.e, shortness of stature, webbing of the neck, scanty or absent axillary and pubic hairs, lower hair line with streak gonad. Chromosomal analysis revealed 45, X/46, XX karyotype. The patient has been well and disease free for a period of 2 years after surgical removal of tumor. Histologically, the tumor was composed of nests of cells arranged in simple and complex annular tubules with central acidophilic hyaline bodies. Ultrastructurally, the cells had deeply indented nuclei and the cells were joined by specialized intercellular junctions along their lateral adjacent borders. Central acidophilic hyaline body consisted of concentric lamellae of basal lamina, but Charcot-Boettcher filaments were not observed.