Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.
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.
Voyadzis
Jean-Marc
JM
Department of Neurosurgery, Georgetown University Medical Center, Washington DC 20007, USA. voyadzij@georgetown.edu
Guttman-Bauman
Ines
I
Santi
Mariarita
M
Cogen
Philip
P
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
D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D006854:Hydrocortisone