Abstract

BEISTI ORTEGO, Anunciación et al. Hypogonadotropic hypogonadism in Klinefelter syndrome and hypothalamic-pituitary tumor. Arch. argent. pediatr. [online]. 2015, vol.113, n.1, pp.e6-e9. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2015.e6.

Klinefelter Syndrome is the most frequent cause of hypergonadotropic hypogonadism in men. A flat response at luteinizing hormone releasing hormone stimulation test could be the first sign of hypothalamic tumor in these patients. We report the case of a patient diagnosed by neonatal screening with Klinefelter Syndrome, 47 XXY, that at 17 years follow-up presents analytical modification of the response to luteinizing hormone releasing hormone stimulation test with suppressed luteinizing hormone and follicle-stimulating hormone values; lately he presents with headache and loss of left eye vision. A magnetic resonance imaging of the brain showed a mixed germ cell hypothalamus tumor, requiring surgery, chemotherapy and radiotherapy with optimal response.

Keywords : Klinefelter Syndrome; Hypogonadism; Germ cell tumors.

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