Pathological satiety caused by brainstem hemangioblastoma.
Because of the multiplicity and saltatory growth pattern associated with central nervous system hemangioblastomas in von Hippel-Lindau (VHL) disease, resection of individual tumors is usually reserved until symptoms occur, to avoid unnecessary surgery over the lifetime of a patient. Brainstem hemangioblastomas in VHL typically cause lower cranial nerve dysfunction, long-tract signs, sensory impairment, and gait abnormalities. The authors report on a 16-year-old girl with VHL who presented with abnormal early satiety resulting in growth and developmental arrest associated with a growing obex hemangioblastoma. Tumor resection resulted in restoration of appetite, with rapid weight gain, growth in stature, and onset of menses. These findings indicate that caudal brainstem-mediated mechanisms have a profound effect on satiety. Moreover, brainstem hemangioblastomas may present with abnormalities in satiety and feeding that can be effectively reversed with resection.
Song
Debbie K
DK
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
Lonser
Russell R
RR
D000293:Adolescent; D049628:Body Size; D020295:Brain Stem Neoplasms / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D002648:Child; D002657:Child Development; D005260:Female; D018325:Hemangioblastoma / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D012528:Satiety Response; D006623:von Hippel-Lindau Disease / Q000473:pathology*