All primary malignant peripheral nerve tumors are derived from the Schwann cell of the neurilemma and should be referred to as malignant neurilemmomas. The Schwann cell is neuroectodermal in origin and retains its embryonic multipotentiality. Epithelial elements, which occur in 10 per cent of these malignant tumors, substantiate this interpretation. A case of a malignant neurilemmoma with epithelial elements is presented. A literature review yields eighteen other cases. The average survival is four years. No generalizations can be made of this tumor except that its innocent clinical presentation, which belies its true malignant nature, necessitates a high index of suspicion by the clinician.
Read full abstract