Extraneural perineurioma

Definition

Extraneural perineurioma is a rare tumor of cranial and spinal nerves arising from peripheral nerve sheet and composed exclusively or predominantly of cells showing perineurial differentiation. It presents as a well-circumscribed, rarely encapsulated mass, not associated with a recognizable nerve, most commonly arising in the dermis and subcutis of the extremities or trunk, or, rarely, in deep soft tissue or skin (e.g., in the stomach, kidney, pancreas, maxillary sinus, mandible, bronchial tree and the face). The clinical presentation depends on the localization.

Also known as soft tissue perineurioma — per MONDO

Also identified as

• NCIT C6912 per MONDO
• Orphanet 100002 per MONDO
• SCTID 768926005 per MONDO
• UMLS C4708595 per MONDO

Affected Organs