Elsevier

Oral Oncology

Volume 135, December 2022, 106211
Oral Oncology

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

https://doi.org/10.1016/j.oraloncology.2022.106211Get rights and content

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.

Introduction

Malignant peripheral nerve sheath tumor (MPNST) is a spindle cell sarcoma accounting for approximately 3–5 % of soft tissue sarcomas [1]. About half of the cases occur in patients with neurofibromatosis type 1 (NF1); others may arise sporadically or in association with radiotherapy [2]. MPNST most commonly affects the trunk and extremities, followed by the head and neck [1], and in rare cases, it can arise centrally within bone [3]. Although radical surgery combined with adjuvant therapy has been applied for decades, patients with MPNST still have poor prognosis with a 5-year overall survival rate of less than 50 % [4]. This study aims to report a rare case of intraosseous MPNST of the maxilla leading to unfavorable outcomes and to review previously reported cases of maxillary MPNST focusing on the prognosis of this aggressive tumor.

Section snippets

Case report

A 38-year-old female presented with pain on the left upper teeth. Her past medical history was unremarkable. A few months before the first visit, the patient had her upper left second premolar and first molar extracted at another hospital due to toothache, but the pain continued. A panoramic radiograph revealed an ill-defined radiolucent lesion in the posterior maxilla with destruction of the inferior and posterior walls of the left maxillary sinus (Figure 1A). Computed tomography showed a

Literature review

After reviewing maxillary MPNST cases reported in the English literature, seven cases with radiological and histological data supporting an intraosseous location and a diagnosis of MPNST, respectively, were included in the current literature analysis (Table 1) [5], [6], [7], [8], [9], [10], [11].

Including the present case, the mean age of maxillary MPNST was 33.5 years (range, 12–65 years), and a male-to-female ratio was 1:1. Of the eight cases investigated, two (25.0 %) occurred in patients

Discussion

Several types of peripheral nerve sheath tumors, including schwannoma, neurofibroma, granular cell tumor, and MPNST, can arise centrally within bone [12], [13]. Intraosseous MPNST can be observed in the jaws [2], spine [14], and bone of the extremities [3], with the most frequent site being the mandible [3], [10]. As seen in the present case, the maxilla can also be affected by MPNST; as discussed previously [12], intraosseous peripheral nerve sheath tumor of the maxilla may arise from one of

Declaration of Competing Interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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