von Hippel–Lindau disease

P Chittiboina, RR Lonser - Handbook of clinical neurology, 2015 - Elsevier
Abstract von Hippel–Lindau (VHL) disease is an inheritable condition with an incidence of 1
in 36 000 live births. Individuals with VHL develop benign and malignant tumors including …

Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphologic, pathologic, surgical features, and outcomes. A systematic review

M Kuharic, D Jankovic, B Splavski, FA Boop… - World neurosurgery, 2018 - Elsevier
Background Posterior cranial fossa (PCF) hemangioblastomas are benign, highly
vascularized, and well-differentiated tumors with well-described histopathologic features …

Microsurgical anatomy of safe entry zones to the brainstem

DD Cavalcanti, MC Preul, MYS Kalani… - Journal of …, 2016 - thejns.org
OBJECT The aim of this study was to enhance the planning and use of microsurgical
resection techniques for intrinsic brainstem lesions by better defining anatomical safe entry …

Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease

RR Lonser, JA Butman, K Huntoon, AR Asthagiri… - Journal of …, 2014 - thejns.org
Object The tumors most frequently associated with von Hippel-Lindau (VHL) disease are
hemangioblastomas. While they are associated with significant neurological impairment and …

Review of the neurological implications of von Hippel–Lindau disease

D Dornbos, HJ Kim, JA Butman, RR Lonser - JAMA neurology, 2018 - jamanetwork.com
Importance von Hippel–Lindau (VHL) disease–associated central nervous system (CNS)
lesions include hemangioblastomas and endolymphatic sac tumors (ELSTs), which are …

Safety and outcomes of preoperative embolization of intracranial hemangioblastomas: a systematic review

L Ampie, W Choy, JB Lamano, K Kesavabhotla… - Clinical Neurology and …, 2016 - Elsevier
Introduction While preoperative embolization is often reserved for large and highly vascular
tumors in order to minimize blood loss, its safety and efficacy in the treatment of …

von Hippel-Lindau syndrome

A Chou, C Toon, J Pickett, AJ Gill - Endocrine Tumor Syndromes and …, 2013 - karger.com
von Hippel-Lindau (VHL) disease is an autosomal-dominant familial cancer syndrome
associated with mutations of the VHL tumor suppressor gene (3p25-26). Its estimated …

Management of von Hippel–Lindau disease-associated CNS lesions

JJ Wind, RR Lonser - Expert review of neurotherapeutics, 2011 - Taylor & Francis
Patients with von Hippel–Lindau disease (VHL) often harbor significant disease burden
within the CNS, specifically craniospinal-axis hemangioblastomas and endolymphatic sac …

Biological and clinical impact of hemangioblastoma-associated peritumoral cysts in von Hippel-Lindau disease

K Huntoon, T Wu, JB Elder, JA Butman, EY Chew… - Journal of …, 2016 - thejns.org
OBJECT Peritumoral cysts are frequently associated with CNS hemangioblastomas and
often underlie neurological morbidity and mortality. To determine their natural history and …

Hemangioblastomas and other vascular origating tumors of brain or spinal cord

IG Vetrano, A Gioppo, G Faragò, V Pinzi, B Pollo… - Human Brain and Spinal …, 2023 - Springer
Hemangioblastomas (HBs) are highly vascularized, slow-growing, rare benigntumors (WHO
grade I). They account for about 2% of intracranialneoplasms; however, they are the most …