Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: Resolution of precocious puberty following microsurgical lesion resection: Case report

Sunil Manjila, Timothy W. Vogel, Yunwei Chen, Mark S. Rodgers, Alan R. Cohen

Research output: Contribution to journalArticlepeer-review

Abstract

Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.

Original languageEnglish (US)
Pages (from-to)101-107
Number of pages7
JournalJournal of Neurosurgery: Pediatrics
Volume14
Issue number1
DOIs
StatePublished - Jul 2014
Externally publishedYes

Keywords

  • Corticotropin-releasing hormone
  • Hypothalamic hamartoma
  • Microsurgery
  • Oncology
  • Precocious puberty
  • Suprasellar arachnoid cyst

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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