Malignant Peripheral Nerve Sheath Tumour
A rare, high-grade soft tissue sarcoma arising from peripheral nerves or exhibiting nerve sheath differentiation, occasionally occurring in the oral and maxillofacial region.
Full Definition
Malignant peripheral nerve sheath tumour (MPNST) is an aggressive sarcoma that may arise sporadically, in association with neurofibromatosis type 1 (NF1), or following therapeutic radiation. In the oral and maxillofacial region, it can involve the mandibular nerve or occur in soft tissues of the cheek and tongue. Histologically, it shows fascicular growth of spindle cells with variable S-100 protein expression, and immunohistochemistry is essential for diagnosis. Editors must ensure that MPNST is not abbreviated before the full term has been defined, and must distinguish it from benign peripheral nerve sheath tumours such as neurofibroma and schwannoma. The abbreviation MPNST is standard in surgical oncology and pathology literature.
Usage
Usage note: The synonyms 'malignant schwannoma' and 'neurogenic sarcoma' are considered outdated; prefer 'malignant peripheral nerve sheath tumour' or MPNST in current documents.
In Context
- "The resection specimen from the posterior mandible was diagnosed as a malignant peripheral nerve sheath tumour in a patient with neurofibromatosis type 1." — Surgical pathology report
- "The author's use of 'malignant schwannoma' was flagged by the editor as outdated nomenclature and replaced with MPNST." — Manuscript editing note