Evidence map›Paper›PMID 26564077›Full record

ReviewHandbook of clinical neurology2015

Von Hippel-Lindau disease.

Prashant Chittiboina, Russell R Lonser

Registry-linked trialOpen access · hybridAbstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Handbook of clinical neurology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07167329 (The BELIEVE-VHL Trial), which is not on this map. Cited by 110 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
110citing papers in PubMed, 4 pooled it
17.3field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT07167329 phase2recruitingnot on this mapstarted 2024, after this paper: background citation

The BELIEVE-VHL Trial: A Real-world Longitudinal Study on Belzutifan's Effectiveness, Pharmacogenetics, and Pharmacoeconomics in Von Hippel-Lindau (VHL) Syndrome Using the HIF2α Inhibitor Belzutifan

TypeinterventionalSponsorJosé Claudio Casali da RochaRan2024 to 2030Enrolled100ConditionsVon Hippel Lindau, Von Hippel Lindau Disease, Von Hippel Lindau-Deficient Clear Cell Renal Cell Carcinoma, Hemangioblastoma (HB) of the Central Nervous System (CNS)ArmsBelzutifan
3 · Its place in the literature

Who cites it

110 citing papers in PubMed, 4 syntheses or guidelines pooled it, 207 citations in OpenAlex.

  1. Guideline
  2. Guideline
  3. Pooled it
  4. Pooled it
  5. Trial
  6. Trial
  7. Belzutifan for von Hippel-Lindau Disease: Pancreatic Lesion Population of the Phase 2 LITESPARK-004 Study.Clinical cancer research : an official journal of the American Association for Cancer Research · 2024
    Trial
  8. Review
  9. Article
  10. Review
  11. Cancer predisposition syndromes: an imaging review.Cancer imaging : the official publication of the International Cancer Imaging Society · 2026
    Review
  12. Review
  13. Article
  14. Ophthalmic screening in phakomatoses.Frontiers in cell and developmental biology · 2026
    Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. Article
  20. Article

50 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

2 authors at 1 institution in 1 country.

Prashant ChittiboinaSurgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA. Electronic address: prashant.chittiboina@nih.gov.
Russell R LonserSurgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA; Department of Neurological Surgery, Ohio State University Wexner Medical Center, Columbus, OH, USA.
National Institute of Neurological Disorders and Stroke · US

Funding

Neuro-oncology of Familial Neoplasia SyndromesZIANS003053 · NINDS · NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE · PI CHITTIBOINA, PRASHANT · 2009 to 2025
$14.9M
Intramural NIH HHS Z99 NS999999
6 · The paper itself

Abstract

von Hippel-Lindau (VHL) disease is an inheritable condition with an incidence of 1 in 36000 live births. Individuals with VHL develop benign and malignant tumors including retinal and central nervous system hemangioblastomas, clear cell renal cell carcinomas (RCC), pheochromocytomas, pancreatic neuroendocrine tumors and endolymphatic sac tumors (ELSTs). VHL is caused by germline loss of function of the VHL gene on one allele at chromosome 3p25-26. A somatic "second hit" event leads to the loss of the other allele and tumor formation. Loss of VHL function in cells leads to increased expression and stabilization of hypoxia inducible factor (HIF). VHL protein/HIF pathway has been implicated in tumorigenesis for hemangioblastomas, RCC and other VHL tumors. Clinical examination, imaging, and genetic testing for VHL mutations confirm VHL disease. Management of VHL disease largely consists of surgical resection of symptomatic tumors (hemangioblastomas), tumors prone to metastasize (RCC larger than 3cm), or tumors causing hormonal symptoms (pheochromocytomas). Despite advances in early diagnosis and management of VHL disease, life expectancy for VHL patients remains low at 40-52 years. Secondary effects from VHL manifestations are mitigated by routine surveillance and early detection. In this chapter, we summarize the current state of knowledge in VHL disease.

Indexed as

Hemangioblastomavon Hippel-Lindau DiseaseHumansclear cell renal cell carcinomaELSTendolymphatic sac tumorhemangioblastomapVHLsecond hitVHLvon Hippel Lindau disease

Identifiers

PMID26564077
PMCPMC5121930
OpenAlexW2414664756

What OpenQuestion holds

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.