Abstract
A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour.
Highlights
Malignant peripheral nerve sheath tumours (MPNSTs) encompass a cluster of tumours which arise from the peripheral nerves or display differentiation along various elements of the nerve sheath, namely, Schwann cells, perineural fibroblasts, or fibroblasts [1]
The MPNSTs are classified as low or high grade based on the cellular differentiation, mitotic count, and expression of immunohistochemistry markers
The cellular staining of MIB-1 proliferation marker in low grade MPNSTs ranges from 1% to 5% [4]
Summary
Malignant peripheral nerve sheath tumours (MPNSTs) encompass a cluster of tumours which arise from the peripheral nerves or display differentiation along various elements of the nerve sheath, namely, Schwann cells, perineural fibroblasts, or fibroblasts [1]. An origin of these tumours in the head and neck region is uncommon occurring in approximately 10% of all cases [2, 3]. A case of maxillary MPNST is discussed along with the literature review of head and neck MPNSTs
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