title: Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.
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.
authors.affiliation: Department of Neurosurgery, Georgetown University Medical Center, Washington DC 20007, USA. voyadzij@georgetown.edu
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Voyadzis
authors.forename: Ines
authors.initials: I
authors.lastname: Guttman-Bauman
authors.forename: Mariarita
authors.initials: M
authors.lastname: Santi
authors.forename: Philip
authors.initials: P
authors.lastname: Cogen
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood; D002653:Child Behavior Disorders / Q000097:blood / Q000209:etiology; D002675:Child, Preschool; D003346:Corticotropin-Releasing Hormone / Q000378:metabolism*; D003907:Dexamethasone; D003937:Diagnosis, Differential; D006222:Hamartoma / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007027:Hypothalamic Diseases / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D007031:Hypothalamus / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male
chemical_list: D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D006854:Hydrocortisone