Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 6, 2023; 11(25): 5910-5918
Published online Sep 6, 2023. doi: 10.12998/wjcc.v11.i25.5910
Clinicopathological study of malignant peripheral nerve sheath tumors in the head and neck: Case reports and review of literature
Long Li, Xiao-Kun Ma, Yan Gao, Dian-Can Wang, Rong-Fang Dong, Jing Yan, Ran Zhang
Long Li, Yan Gao, Jing Yan, Ran Zhang, 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
Long Li, Yan Gao, Jing Yan, Ran Zhang, Research Unit of Precision Pathologic Diagnosis in Tumors of the Oral and Maxillofacial Regions, Chinese Academy of Medical Sciences, Beijing 100000, China
Long Li, Department of Oral Pathology, Xiangya Stomalogical Hospital & School of Stomatology, Central South University, Changsha 410078, Hunan Province, China
Xiao-Kun Ma, Department of Oral and Maxillofacial Surgery, The Affiliated Stomatological Hospital of Soochow University, Suzhou Stomatological Hospital, Suzhou 215000, Jiangsu Province, China
Dian-Can Wang, Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology, Peking University School and Hospital of Stomatology, Beijing 100000, China
Rong-Fang Dong, Department of Pathology, Beijing Jishuitan Hospital, Beijing 100000, China
Author contributions: Li L and Ma XK contributed equally to this work; Zhang R, Li L, Ma XK, Wang DC, Gao Y designed the research study; Jing Y, Dong RF performed the research; Li L, Ma XK, Zhang R analyzed the data and wrote the manuscript; All authors have read and approve the final manuscript.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All the authors declare that they have no conflicts of interest related to this manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ran Zhang, MD, Assistant Professor, 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, No. 22 South Zhongguancun Avenue, Haidian District, Beijing 100081, China. rani@bjmu.edu.cn
Received: March 27, 2023
Peer-review started: March 27, 2023
First decision: July 3, 2023
Revised: July 20, 2023
Accepted: August 8, 2023
Article in press: August 8, 2023
Published online: September 6, 2023
Processing time: 157 Days and 21.6 Hours
Abstract
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% and 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.

Keywords: Malignant peripheral nerve sheath tumor; Head and neck; Treatment; Intraosseous; Surgery; Case report

Core Tip: We retrospectively reviewed patients with head and neck malignant peripheral nerve sheath tumors treated in our hospital from 2000 to 2021. The study population consisted of five women and five men aged 22–75 years (mean age, 49 years). The 2- and 5-year disease-specific survival rates were 86% and 43%, respectively. The average survival time was 64 mo. 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).