504 EARLY PUBERTY IN KLINEFELTER SYNDROME DUE TO A CHORIONIC GONADOTROPIN (Hcg)-Producing TUMO

A 15 year old white male presented with a 3-week history of productive cough, hemoptysis and weight loss. His past history revealed the presence of pubic hair at age 10 and pubertal growth spurt at 12 years of age. physical examination revealed pubic hair stage V, axillary and facial hair stage III. Penile length was 12 cm and testicular volume 4 ml. The small size of the testes led to determination of karotype, which was 47,XXY. Chest X-ray revealed opacification of the right hemithorax. CT scan of the chest showed a large heterogeneous density with solid, fluid and calcified areas. Mediastinal shift to the left was noted. Thoracotomy was performed, and biopsied tissue revealed an immature Teratoma with histologic characteristics of malignancy. Serum Alpha-Fetoprotein (16.600 mcg/L), hCG (2,810 U/L) and LH (125 mIU/ul) were found to be greatly elevated. Serum FSH (8 mIU/ml) and testosterone (623 ng%) were normal. Probably the early puberty seen in this patient resulted from the secretion of hCG, from the thoracic teratoma. This patient as well as the others reported in the literature demonstrate that the association of a gonadotropin-secreting teratoma with Klinefelter syndrome must be more than accidental.