Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis
Abstract Introduction Malignant peripheral nerve sheath tumors (MPNSTs) are malignancies that demonstrate nerve sheath differentiation in the peripheral nervous system. They can occur sporadically or be associated with neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, wi...
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| Format: | Article |
| Language: | English |
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BMC
2024-01-01
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| Series: | World Journal of Surgical Oncology |
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| Online Access: | https://doi.org/10.1186/s12957-023-03296-z |
| _version_ | 1827382139823325184 |
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| author | Zhixue Lim Tian Yuan Gu Bee Choo Tai Mark Edward Puhaindran |
| author_facet | Zhixue Lim Tian Yuan Gu Bee Choo Tai Mark Edward Puhaindran |
| author_sort | Zhixue Lim |
| collection | DOAJ |
| description | Abstract Introduction Malignant peripheral nerve sheath tumors (MPNSTs) are malignancies that demonstrate nerve sheath differentiation in the peripheral nervous system. They can occur sporadically or be associated with neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, with up to 13% of patients developing MPNSTs in their lifetimes. Previous studies have suggested conflicting findings regarding the prognosis of NF1 for patients with MPNSTs. The elucidation of NF1 as an independent prognostic factor on mortality has implications for clinical management. We aim to investigate the role of NF1 status as an independent prognostic factor of overall survival (OS) and disease-specific survival (DSS) in MPNSTs. Methods An electronic literature search of PubMed and MEDLINE was performed on studies reporting OS or DSS outcomes of MPNSTs with and without NF1. A grey literature search by reviewing bibliographies of included studies and review articles was performed to find pertinent studies. Data was extracted and assessed in accordance with the PRISMA guidelines. A meta-analysis was performed to calculate hazard ratios (HRs) using a random-effects model. The primary and secondary outcomes were all-cause and disease-specific mortality, respectively, with NF1 as an independent prognostic factor of interest. Results A total of 59 retrospective studies involving 3602 patients fulfilled the inclusion criteria for OS analysis, and 23 studies involving 704 MPNST patients were included to evaluate DSS outcomes. There was a significant increase in the hazard of all-cause mortality (HR 1.63, 95% CI 1.45 to 1.84) and disease-specific mortality (HR 1.52, 95% CI 1.24 to 1.88) among NF1 as compared to sporadic cases. Subgroup analyses and meta-regression showed that this result was consistent regardless of the quality of the study and year of publication. Conclusion NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality. This finding suggests that closer surveillance is required for NF1 patients at risk of developing MPNSTs. |
| first_indexed | 2024-03-08T14:15:15Z |
| format | Article |
| id | doaj.art-77fa2f4301794a5b9abdb45cc877b8ca |
| institution | Directory Open Access Journal |
| issn | 1477-7819 |
| language | English |
| last_indexed | 2024-03-08T14:15:15Z |
| publishDate | 2024-01-01 |
| publisher | BMC |
| record_format | Article |
| series | World Journal of Surgical Oncology |
| spelling | doaj.art-77fa2f4301794a5b9abdb45cc877b8ca2024-01-14T12:25:23ZengBMCWorld Journal of Surgical Oncology1477-78192024-01-0122111410.1186/s12957-023-03296-zSurvival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysisZhixue Lim0Tian Yuan Gu1Bee Choo Tai2Mark Edward Puhaindran3Department of Hand & Reconstructive Microsurgery, University Orthopaedic, Hand & Reconstructive Microsurgical Cluster, National University Health SystemSaw Swee Hock School of Public Health, National University of Singapore and National University Health SystemSaw Swee Hock School of Public Health, National University of Singapore and National University Health SystemDepartment of Hand & Reconstructive Microsurgery, University Orthopaedic, Hand & Reconstructive Microsurgical Cluster, National University Health SystemAbstract Introduction Malignant peripheral nerve sheath tumors (MPNSTs) are malignancies that demonstrate nerve sheath differentiation in the peripheral nervous system. They can occur sporadically or be associated with neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, with up to 13% of patients developing MPNSTs in their lifetimes. Previous studies have suggested conflicting findings regarding the prognosis of NF1 for patients with MPNSTs. The elucidation of NF1 as an independent prognostic factor on mortality has implications for clinical management. We aim to investigate the role of NF1 status as an independent prognostic factor of overall survival (OS) and disease-specific survival (DSS) in MPNSTs. Methods An electronic literature search of PubMed and MEDLINE was performed on studies reporting OS or DSS outcomes of MPNSTs with and without NF1. A grey literature search by reviewing bibliographies of included studies and review articles was performed to find pertinent studies. Data was extracted and assessed in accordance with the PRISMA guidelines. A meta-analysis was performed to calculate hazard ratios (HRs) using a random-effects model. The primary and secondary outcomes were all-cause and disease-specific mortality, respectively, with NF1 as an independent prognostic factor of interest. Results A total of 59 retrospective studies involving 3602 patients fulfilled the inclusion criteria for OS analysis, and 23 studies involving 704 MPNST patients were included to evaluate DSS outcomes. There was a significant increase in the hazard of all-cause mortality (HR 1.63, 95% CI 1.45 to 1.84) and disease-specific mortality (HR 1.52, 95% CI 1.24 to 1.88) among NF1 as compared to sporadic cases. Subgroup analyses and meta-regression showed that this result was consistent regardless of the quality of the study and year of publication. Conclusion NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality. This finding suggests that closer surveillance is required for NF1 patients at risk of developing MPNSTs.https://doi.org/10.1186/s12957-023-03296-zMalignant peripheral nerve sheath tumorsSarcomaNeurofibromatosisSurvivalOutcomesMortality |
| spellingShingle | Zhixue Lim Tian Yuan Gu Bee Choo Tai Mark Edward Puhaindran Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis World Journal of Surgical Oncology Malignant peripheral nerve sheath tumors Sarcoma Neurofibromatosis Survival Outcomes Mortality |
| title | Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis |
| title_full | Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis |
| title_fullStr | Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis |
| title_full_unstemmed | Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis |
| title_short | Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis |
| title_sort | survival outcomes of malignant peripheral nerve sheath tumors mpnsts with and without neurofibromatosis type i nf1 a meta analysis |
| topic | Malignant peripheral nerve sheath tumors Sarcoma Neurofibromatosis Survival Outcomes Mortality |
| url | https://doi.org/10.1186/s12957-023-03296-z |
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