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Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection
Published on: April 12, 2024
Pathological satiety caused by brainstem hemangioblastoma.
Debbie K Song1, Russell R Lonser
1Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
Journal of Neurosurgery. Pediatrics
|November 28, 2008
Summary
Central nervous system hemangioblastomas in von Hippel-Lindau (VHL) disease can cause satiety issues. Surgical removal of these brainstem tumors can reverse feeding abnormalities and promote growth.
Area of Science:
- Neuro-oncology
- Genetics
- Pediatric Endocrinology
Background:
- Von Hippel-Lindau (VHL) disease is a genetic disorder characterized by the development of central nervous system hemangioblastomas.
- Tumor resection is typically delayed until symptoms arise due to tumor multiplicity and growth patterns.
Observation:
- A 16-year-old female with VHL presented with early satiety, leading to growth and developmental arrest.
- The patient had a growing hemangioblastoma located in the obex region of the brainstem.
Findings:
- Surgical resection of the obex hemangioblastoma led to the restoration of appetite.
- Post-operatively, the patient experienced rapid weight gain, increased stature, and menarche.
Implications:
- Caudal brainstem mechanisms significantly influence satiety regulation.
- Brainstem hemangioblastomas in VHL disease can manifest as feeding and satiety abnormalities, which are reversible with surgical intervention.
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