ArticleJournal of neuro-oncology2007
Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors.
Article in Journal of neuro-oncology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 2 of them syntheses that pooled it.
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Who cites it
25 citing papers in PubMed, 2 syntheses or guidelines pooled it, 93 citations in OpenAlex.
- Survival and prognosis of neurofibromatosis type 1-associated malignant peripheral nerve sheath tumours: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2026Pooled it
- Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis.World journal of surgical oncology · 2024Pooled it
- Early Detection of Malignant and Premalignant Peripheral Nerve Tumors Using Cell-Free DNA Fragmentomics.Clinical cancer research : an official journal of the American Association for Cancer Research · 2024Article
- Early detection of malignant and pre-malignant peripheral nerve tumors using cell-free DNA fragmentomics.medRxiv : the preprint server for health sciences · 2024Article
- A worldwide bibliometric analysis of malignant peripheral nerve sheath tumors from 2000 to 2022.Frontiers in oncology · 2023Article
- Case series of congenital pseudarthrosis of the tibia unfulfilling neurofibromatosis type 1 diagnosis: 21% with somatic NF1 haploinsufficiency in the periosteum.Human genetics · 2022Article
- Article
- Aberrant ATRX protein expression is associated with poor overall survival in NF1-MPNST.Oncotarget · 2018Article
- Prospect and progress of oncolytic viruses for treating peripheral nerve sheath tumors.Expert opinion on orphan drugs · 2016Article
- Intrathoracic Malignant Peripheral Nerve Sheath Tumor: Histopathological and Immunohistochemical Features.Iranian journal of pathology · 2015Article
- Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor.Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2014Article
- Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.Frontiers in oncology · 2014Article
- Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1.Neuro-oncology · 2013Review
- Genomic imbalance of HMMR/RHAMM regulates the sensitivity and response of malignant peripheral nerve sheath tumour cells to aurora kinase inhibition.Oncotarget · 2013Article
- Article
- Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study.Orphanet journal of rare diseases · 2012Article
- A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion.Journal of neuro-oncology · 2012Article
- Podoplanin and CD34 in peripheral nerve sheath tumours: focus on neurofibromatosis 1-associated atypical neurofibroma.Journal of neuro-oncology · 2011Article
- Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five years after diagnosis: a rare finding.International journal of clinical and experimental pathology · 2010Article
- Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors.Neuro-oncology · 2009Article
Corrections and comments
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Authors and funding
9 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively. The collective comprised 38 NF1 patients and 14 sporadic patients. NF1 patients were significantly younger at diagnosis (p<0.001) and had a significantly shorter survival time than sporadic patients (median survival 17 months vs. 42 months, Breslow p<0.05). The time interval to local recurrence and metastatic spread was also significantly shorter in NF1 patients (9.4 months vs. 30.0 months, p<0.01; 9.1 months vs. 33.2 months, p<0.001, respectively). In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p<0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p<0.01). Most importantly, while histopathological variables correlated with French Fédération Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors. The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups. Our findings indicate the necessity for a separate grading scheme which takes into account the genetic background in NF1 patients.
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