Evidence map›Paper›PMID 38647646›Full record

ArticleJournal of neuro-oncology2024

Screening and surveillance recommendations for central nervous system hemangioblastomas in pediatric patients with Von Hippel-Lindau disease.

Anna Laura Knoblauch, B-I Blaß, C Steiert, N Neidert, A Puzik, E Neumann-Haefelin, A Ganner, F Kotsis, T Schäfer, H P H Neumann and 3 more

Open access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of neuro-oncology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
1.4field-weighted citation impact, top 19% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed, 5 citations in OpenAlex.

  1. Review
  2. Article
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  4. Review
  5. Article
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

13 authors at 1 institution in 1 country.

Anna Laura KnoblauchDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. annalaura.knoblauch@web.de.
B-I BlaßDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
C SteiertDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
N NeidertDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
A PuzikDepartment of Pediatric Hematology and Oncology, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
E Neumann-HaefelinRenal Division, Department of Medicine, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
A GannerRenal Division, Department of Medicine, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
F KotsisRenal Division, Department of Medicine, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
T SchäferRenal Division, Department of Medicine, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
H P H NeumannRenal Division, Department of Medicine, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
S ElsheikhDepartment of Neuroradiology, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Freiburg, Germany.
J BeckDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
J-H KlinglerDepartment of Neurosurgery, Faculty of Medicine, Medical Center - University of Freiburg, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
University of Freiburg · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeVon Hippel-Lindau (VHL) disease is an autosomal-dominantly inherited tumor predisposition syndrome. One of the most common tumors are central nervous system (CNS) hemangioblastomas. Recommendations on the initiation and continuation of the screening and surveillance program for CNS tumors in pediatric VHL patients are based on small case series and thus low evidence level. To derive more robust screening recommendations, we report on the largest monocentric pediatric cohort of VHL patients.

methodsWe performed a retrospective analysis on a pediatric cohort of 99 VHL patients consulted at our VHL center from 1992 to 2023. Clinical, surgical, genetic, and imaging data were collected and statistically analyzed.

results42 patients (50% male) developed CNS hemangioblastomas, of whom 18 patients (56% male) underwent hemangioblastoma surgery (mean age at first surgery: 14.9 ± 1.9 years; range 10.2-17). The first asymptomatic patient was operated on at the age of 13.2 years due to tumor progress. Truncating VHL mutation carriers had a significantly higher manifestation rate (HR = 3.7, 95% CI: 1.9-7.4, p < 0.0001) and surgery rate (HR = 3.3, 95% CI: 1.2-8.9, p = 0.02) compared with missense mutation carriers.

conclusionWe recommend starting MRI imaging at the age of 12 years with examination intervals every (1-) 2 years depending on CNS involvement. Special attention should be paid to patients with truncating variants. Affected families should be educated regularly on potential tumor-associated symptoms to enable timely MRI imaging and eventually intervention, as CNS hemangioblastoma may develop before screening begins. GERMAN CLINICAL TRIALS REGISTER REGISTRATION NUMBER: DRKS00029553, date of registration 08/16/2022, retrospectively registered.

Indexed as

Hemangioblastomavon Hippel-Lindau DiseaseAdolescentCentral Nervous System NeoplasmsCerebellar NeoplasmsChildFemaleFollow-Up StudiesHumansMaleRetrospective StudiesVon Hippel-Lindau Tumor Suppressor ProteinVon Hippel-Lindau Tumor Suppressor ProteinAdolescenceHemangioblastomaPediatric neurosurgerySurveillanceVon Hippel-Lindau

Identifiers

PMID38647646
PMCPMC11186940
OpenAlexW4394998123

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.