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
Volume100
Issue2 Suppl Pediatrics
Pagination212-6
Date Published2004 Feb
ISSN0022-3085
KeywordsAdrenocorticotropic Hormone, Child Behavior Disorders, Child, Preschool, Corticotropin-Releasing Hormone, Dexamethasone, Diagnosis, Differential, Hamartoma, Humans, Hydrocortisone, Hypothalamic Diseases, hypothalamus, Magnetic Resonance Imaging, Male
Abstract

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.

DOI10.1111/j.1708-8208.2012.00444.x
Alternate JournalJ. Neurosurg.