Malignant peripheral nerve sheath tumor of the maxilla: Case report and review of the literature with emphasis on its poor prognosis. (December 2022)

• Record Type: Journal Article
• Authors: Oh, Kyu-Young; Hong, Seong-Doo
• Is Part Of: Oral oncology. Volume 135(2022)
• Publication Date: 2022-12
• Subjects: Malignant peripheral nerve sheath tumor -- Maxilla -- Intraosseous -- Prognosis; Mouth -- Cancer -- Periodicals; Mouth -- Tumors -- Periodicals; Mouth Diseases -- Periodicals; Mouth Neoplasms -- Periodicals; Bouche -- Cancer -- Périodiques; Bouche -- Tumeurs -- Périodiques; Tumeurs -- Périodiques; Electronic journals; 616.9943105
• DOI: 10.1016/j.oraloncology.2022.106211
• Languages: English
• ISSNs: 1368-8375

Abstract

Abstract: Malignant peripheral nerve sheath tumor (MPNST) is a spindle cell sarcoma with poor prognosis. Although patients with neurofibromatosis type 1 (NF1) have a higher risk of MPNST, it can also occur in the sporadic setting and may rarely arise centrally within bone. In this study, we present an extremely rare case of intraosseous MPNST of the maxilla arising in a 38-year-old female with no history of NF1. Despite radical surgery and postoperative radiotherapy, the patient died due to multiple distant metastases 1 year after treatment. According to the results of the literature analysis performed in this study, maxillary MPNST cases have worse clinical outcomes than general MPNSTs. In addition, it seems that NF1 and histological necrosis are poor prognostic indicators in patients with maxillary MPNST.