Synonyms: Malignant perineurioma
Malignant peripheral nerve sheath tumor with perineurial differentiation is a rare soft tissue sarcoma composed predominantly of spindle-shaped neoplastic cells showing perineurial differentiation and displaying abundant cellular pleomorphism or anaplasia frequent mitoses tumor necrosis and high metastatic potential. It often presents as a soft painless solid mass in subcutaneous tissues of the trunk or limbs but tumors have also been described in the facial area mediastinum retroperitoneum pancreas paravertebral column and the pelvic soft tissues. Frequent local recurrence and distant metastatic spread has been reported.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2026
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