Clinicopathological study of malignant peripheral nerve sheath tumors in the head and neck:Case reports and review of literature  

作　　者：Long Li Xiao-Kun Ma Yan Gao Dian-Can Wang Rong-Fang Dong Jing Yan Ran Zhang 

机构地区：[1]Department of Oral Pathology,Peking University School and Hospital of Stomatology,National Clinical Research Center for Oral Diseases,National Engineering Laboratory for Digital and Material Technology of Stomatology,Beijing Key Laboratory of Digital Stomatology,Beijing 100000,China [2]Research Unit of Precision Pathologic Diagnosis in Tumors of the Oral and Maxillofacial Regions,Chinese Academy of Medical Sciences,Beijing 100000,China [3]Department of Oral Pathology,Xiangya Stomalogical Hospital&School of Stomatology,Central South University,Changsha 410078,Hunan Province,China [4]Department of Oral and Maxillofacial Surgery,The Affiliated Stomatological Hospital of Soochow University,Suzhou Stomatological Hospital,Suzhou 215000,Jiangsu Province,China [5]Department of Oral and Maxillofacial Surgery,Peking University School and Hospital of Stomatology,Peking University School and Hospital of Stomatology,Beijing 100000,China [6]Department of Pathology,Beijing Jishuitan Hospital,Beijing 100000,China

出　　处：《World Journal of Clinical Cases》2023年第25期5910-5918,共9页世界临床病例杂志

摘　　要：BACKGROUND Malignant peripheral nerve sheath tumor(MPNST)is a rare and aggressive soft tissue sarcoma that poses a major diagnostic and therapeutic challenge.CASE SUMMARY We retrospectively reviewed patients with head and neck MPNSTs treated in our hospital from 2000 to 2021.The clinical features,pathological manifestations,treatments,and prognoses were summarized.We also reviewed the literature,focusing on MPNST in the mandible and maxilla.The study population consisted of five women and five men aged 22–75 years(mean age,49 years).Of the 10 patients,7 were initial cases and 3 were recurrent cases.All lesions were sporadic.The most common site was the mandible.The most frequently encountered symptoms were a progressive mass and local swelling.Complete or partial loss of trimethylation at lysine 27 of histone H3(H3K27me3)was evident on staining in four of nine cases(one case was excluded due to lack of tissue for evaluation of loss of H3K27me3).The 2-and 5-year disease-specific survival rates were 86%a nd 43%,respectively.The average survival time was 64 mo.CONCLUSION MPNST is a highly malignant tumor with a poor prognosis,prone to a high risk of recurrence and distant metastasis.Complete surgical resection is the main treatment.

关 键 词：Malignant peripheral nerve sheath tumor Head and neck TREATMENT INTRAOSSEOUS SURGERY Case report 

分 类 号：R739.4[医药卫生—肿瘤] R739.5[医药卫生—临床医学]