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Updated: Aug 29, 2026

Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection
Published on: April 12, 2024
Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report
Jean-Marc Voyadzis1, Ines Guttman-Bauman, Mariarita Santi
1Department of Neurosurgery, Georgetown University Medical Center, Washington DC 20007, USA. voyadzij@georgetown.edu
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.
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