Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.

TitleHypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.
Publication TypeJournal Article
Year of Publication2004
AuthorsVoyadzis, J-M, Guttman-Bauman I, Santi M, Cogen P
JournalJournal of neurosurgery
Issue2 Suppl Pediatrics
Date Published2004 Feb
KeywordsAdrenocorticotropic Hormone, Child Behavior Disorders, Child, Preschool, Corticotropin-Releasing Hormone, Dexamethasone, Diagnosis, Differential, Hamartoma, Humans, Hydrocortisone, Hypothalamic Diseases, hypothalamus, Magnetic Resonance Imaging, Male

The authors describe a unique case of a 2-year-old boy with a hypothalamic hamartoma secreting corticotropin-releasing hormone (CRH). The patient presented with a history of behavioral disturbances progressing over 12 months. His neurological status was intact. Magnetic resonance imaging demonstrated a 1.8 x 1.6 x 1.2-cm isointense, nonenhancing hypothalamic lesion. Endocrinological workup revealed elevated serum CRH and adrenocorticotropic hormone levels, nonsuppression with low-dose dexamethasone, and partial suppression with high-dose dexamethasone. He underwent tumor resection via a right frontotemporal craniotomy. Pathological examination of the tissue confirmed a hypothalamic hamartoma with CRH immunostaining. Postoperatively, his hormone levels normalized and his behavioral disturbances abated. The radiographic and clinical characteristics of hypothalamic hamartomas are reviewed and therapeutic considerations discussed.

Alternate JournalJ. Neurosurg.