Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.
|Title||Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report.|
|Publication Type||Journal Article|
|Year of Publication||2004|
|Authors||Voyadzis, J-M, Guttman-Bauman I, Santi M, Cogen P|
|Journal||Journal of neurosurgery|
|Issue||2 Suppl Pediatrics|
|Date Published||2004 Feb|
|Keywords||Adrenocorticotropic 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 Journal||J. Neurosurg.|