von Hippel–Lindau disease: update on pathogenesis and systemic aspects

ME Aronow, HE Wiley, A Gaudric, V Krivosic, MB Gorin… - Retina, 2019 - journals.lww.com
Purpose: To provide an update summarizing the biologic pathways governing von Hippel–
Lindau (VHL) disease pathogenesis and to provide an overview of systemic manifestations …

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 …

Genetic analysis of von Hippel‐Lindau disease

M Nordstrom‐O'Brien, RB van der Luijt… - Human …, 2010 - Wiley Online Library
Mutations in the von Hippel-Lindau (VHL) gene are responsible for VHL disease, congenital
polycythemia, and are found in many sporadic tumor types as well. Reports of VHL …

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 …

Neurologic manifestations of von Hippel-Lindau disease

JA Butman, WM Linehan, RR Lonser - Jama, 2008 - jamanetwork.com
von Hippel-Lindau disease (VHL) is an autosomal-dominant neoplasia syndrome that is the
result of a germline mutation of the VHL tumor suppressor gene on the short arm of …

Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel–Lindau disease

AR Asthagiri, GU Mehta, L Zach, X Li… - Neuro …, 2010 - academic.oup.com
To determine the effectiveness of stereotactic radiosurgery (SRS) treatment to central
nervous system (CNS) hemangioblastomas in von Hippel–Lindau disease (VHL), we …

Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study

H Kano, T Shuto, Y Iwai, J Sheehan… - Journal of …, 2015 - thejns.org
OBJECT The purpose of this study was to evaluate the role of stereotactic radiosurgery
(SRS) in the management of intracranial hemangioblastomas. METHODS Six participating …

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 …

Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment

JH Klingler, S Gläsker, B Bausch, H Urbach… - Child's Nervous …, 2020 - Springer
Introduction Hemangioblastomas are rare, histologically benign, highly vascularized tumors
of the brain, the spinal cord, and the retina, occurring sporadically or associated with the …

Incidence, prognostic factors and survival for hemangioblastoma of the central nervous system: analysis based on the surveillance, epidemiology, and end results …

X Yin, H Duan, Z Yi, C Li, R Lu, L Li - Frontiers in Oncology, 2020 - frontiersin.org
Background Hemangioblastomas are uncommon, benign neoplasms of the central nervous
system (CNS). This study aims to evaluate the incidence, demographics, clinical …