Klinefelter’s Syndrome with Retroperitoneal Teratoma Masquerading as Adrenal Mass: A Very Rare Occurrence in Adults

The association of chromosomal disorders with variousneoplasms is well established. We report a case of 36 yearsold man who was referred for preoperative evaluation offunctional status of “presumed” right adrenal mass. Thepatient was found to have clinical features of hypogonadismand further investigations confirmed the diagnosis ofKlinefelter’s Syndrome. The patient proceeded to surgeryand pathological examination of the removed mass revealeda mature retroperitoneal teratoma abutting the adrenalgland. This case demonstrates that patients with KS are atincreased risk of extragonadal germ cell tumors unusualsites from the mediastinum and brain. Retroperitonealteratoma in the adrenal gland region can be easily confusedwith adrenal related mass. Key words: Klinefelter’s syndrome, Adrenal incidentilomas,Hypogonadotrophic Hypogonadism, Retroperitonealteratoma.