Hypothalamic hamartoma secreting corticotropin-releasing hormone: Case report

Jean Marc Voyadzis, Ines Guttman-Bauman, Mariarita Santi, Philip Cogen

    Research output: Contribution to journalArticle

    9 Scopus citations

    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.

    Original languageEnglish (US)
    Pages (from-to)212-216
    Number of pages5
    JournalJournal of neurosurgery
    Volume100
    Issue number2 SUPPL.
    StatePublished - Feb 1 2004

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    Keywords

    • Corticotropin-releasing hormone
    • Cushing disease
    • Endocrinology
    • Hamartoma
    • Hypothalamus
    • Pediatric neurosurgery

    ASJC Scopus subject areas

    • Surgery
    • Clinical Neurology

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