Gi Jeong Park, Sang Hoon Kim, K. Kim, Dae-Hyun Kim
{"title":"Different Pathology of Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1: 3 Cases","authors":"Gi Jeong Park, Sang Hoon Kim, K. Kim, Dae-Hyun Kim","doi":"10.21129/nerve.2021.7.2.92","DOIUrl":null,"url":null,"abstract":"Neurofibromatosis type 1 (NF1) is an autosomal-dominant genetic disease that predisposes affected individuals to tumors. Neurofibroma and malignant peripheral nerve sheath tumor (MPNST) are examples of PNSTs that occur either sporadically or as part of hereditary neurocutaneous diseases such as NF1. We treated three patients with NF1 who presented with different PNSTs. All patients underwent surgical resection, and pathologic findings indicated neurofibroma, atypical neurofibroma, and MPNST, respectively. We managed each case based on its pathology. The patient with MPNST died after chemoradiotherapy; the other patients did not experience recurrence for several months.","PeriodicalId":229172,"journal":{"name":"The Nerve","volume":"s3-27 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2021-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Nerve","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.21129/nerve.2021.7.2.92","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Neurofibromatosis type 1 (NF1) is an autosomal-dominant genetic disease that predisposes affected individuals to tumors. Neurofibroma and malignant peripheral nerve sheath tumor (MPNST) are examples of PNSTs that occur either sporadically or as part of hereditary neurocutaneous diseases such as NF1. We treated three patients with NF1 who presented with different PNSTs. All patients underwent surgical resection, and pathologic findings indicated neurofibroma, atypical neurofibroma, and MPNST, respectively. We managed each case based on its pathology. The patient with MPNST died after chemoradiotherapy; the other patients did not experience recurrence for several months.