Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection.
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.
Division of Pediatric Neurosurgery, Rainbow Babies and Children's Hospital, Department of Neurological Surgery, Neurological Institute;
Sunil
S
Manjila
Timothy W
TW
Vogel
Yunwei
Y
Chen
Mark S
MS
Rodgers
Alan R
AR
Cohen
D016080:Arachnoid Cysts / Q000175:diagnosis*; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications* / Q000175:diagnosis*; D007223:Infant; D008279:Magnetic Resonance Imaging; D008866:Microsurgery; D044583:Neuroendoscopy; D019635:Neurosurgical Procedures / Q000379:methods*; D011629:Puberty, Precocious / Q000209:etiology*; D016896:Treatment Outcome
CRH = corticotropin-releasing hormone; GFAP = glial fibrillary acidic protein; HH = hypothalamic hamartoma; LH = luteinizing hormone; corticotropin-releasing hormone; hypothalamic hamartoma; microsurgery; oncology; precocious puberty; suprasellar arachnoid cyst