Evidence map›Paper›PMID 26851632›Full record

ReviewNeuro-oncology2016

Therapeutic advances for the tumors associated with neurofibromatosis type 1, type 2, and schwannomatosis.

Jaishri O Blakeley, Scott R Plotkin

2 registry-linked trialsAbstract readReview
In one paragraph

Review in Neuro-oncology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 72 papers.

0numbers the graph read from it
0cells of the map it votes in
72citing papers in PubMed
–field-weighted citation impact
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.

NCT06620354 phase2not yet recruitingnot on this mapstarted 2024, after this paper: background citation

Prospective, One-arm, Phase II Clinical Study of Smeitinib Hydrosulfate Capsules for the Treatment of Patients With Type I Neurofibromatosis

TypeinterventionalSponsorSun Yat-Sen Memorial Hospital of Sun Yat-Sen UniversityRan2024 to 2026Enrolled33ConditionsNeurofibromatosis 1ArmsSelumetinib
NCT06621082 phase2not yet recruitingnot on this mapstarted 2024, after this paper: background citation

A Prospective, One-arm, Phase II Clinical Study of Smeitinib Hydrosulfate Capsules for the Treatment of Patients With Type I Neurofibromatosis After Surgery

TypeinterventionalSponsorSun Yat-Sen Memorial Hospital of Sun Yat-Sen UniversityRan2024 to 2026Enrolled19ConditionsNeurofibromatosis 1ArmsSelumetinib
3 · Its place in the literature

Who cites it

72 citing papers in PubMed.

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  17. [A case report of giant neurofibromatosis of maxillofacial, neck and chest was treated by multidisciplinary cooperation].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2025
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12 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.

Jaishri O BlakeleyNeurology, Neurosurgery and Oncology, Johns Hopkins University, Baltimore, MD (J.O.B.); Neurology, Harvard Medical School, Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital, Boston, MA (S.R.P.).
Scott R PlotkinNeurology, Neurosurgery and Oncology, Johns Hopkins University, Baltimore, MD (J.O.B.); Neurology, Harvard Medical School, Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital, Boston, MA (S.R.P.).

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN) are tumor-suppressor syndromes. Each syndrome is an orphan disease; however, the tumors that arise within them represent the most common tumors of the nervous system worldwide. Systematic investigation of the pathways impacted by the loss of function of neurofibromin (encoded byNF1) and merlin (encoded byNF2) have led to therapeutic advances for patients with NF1 and NF2. In the syndrome of SWN, the genetic landscape is more complex, with 2 known causative genes (SMARCB1andLZTR1) accounting for up to 50% of familial SWN patients. The understanding of the molecular underpinnings of these syndromes is developing rapidly and offers more therapeutic options for the patients. In addition, common sporadic cancers harbor somatic alterations inNF1(ie, glioblastoma, breast cancer, melanoma),NF2(ie, meningioma, mesothelioma) andSMARCB1(ie, atypical teratoid/rhabdoid tumors) such that advances in management of syndromic tumors may benefit patients both with and without germline mutations. In this review, we discuss the clinical and genetic features of NF1, NF2 and SWN, the therapeutic advances for the tumors that arise within these syndromes and the interaction between these rare tumor syndromes and the common tumors that share these mutations.

Indexed as

HumansMedical OncologyNeurilemmomaNeurofibromatosesNeurofibromatosis 1Neurofibromatosis 2Skin NeoplasmsNF1NF2schwannomatosistherapeuticstumor suppressor syndrome

Identifiers

PMID26851632
PMCPMC4827037

What OpenQuestion holds

Textmetadata
Read underepoch 390

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.