SUPRASELLAR TUMORS OF MALDEVELOPMENTAL ORIGIN IN KLINEFELTERS-SYNDROME - A REPORT OF 2 CASES

Patients with Klinefelter's syndrome may have a predisposition for the development of neoplasia, particularly extragonadal germ-cell tumors, but a suprasellar location is rarely reported. The clinical and neuroradiologic features in two patients with Klinefelter's syndrome and dysmorphic suprasellar masses of maldevelopmental origin (presumably lipomas or lipodermoids) are described. One patient had bilateral optic atrophy and decreased vision. To our knowledge, only one similar case (a suprasellar hamartoma) has been previously reported in association with Klinefelter's syndrome.