Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (von Recklinghausen's disease)

Abstract

M multiple neurofibromatosis include gliomas of the optic nerve, astrocytomas of the cerebral and cerebellar hemispheres, intramedullary gliomas of the spinal cord, and sarcomas of peripheral nerves and somatic soft tissues.This paper concerns the problems of diagnosis, classification, and management of sarcomas that arise in anatomically discernible nerves or somatic soft tissues in association with multiple neurofibromatosis.Many investigators have, perhaps more or less arbitrarily, designated these tumors as malignant neurilemomas (neurogenic sarcoma, neurofibrosarcoma, malignant schwannoma), thereby implying directly or indirectly that the neurilemmal cell is the cell of origin.As in the case of primary malignant tumors of nerve not associated with multiple neurofibromatosis, the specificity of the microscopic pattern is not necessarily conclusive evidence for distinguishing malignant neurilemomas from fibrosarcomas or other mesenchymal sarcomas.6In patients exhibiting the manifestations of multiple neurofibromatosis, malignant tumors, apparently arising in the somatic soft tissues independently of nerves, are encountered, in addition to malignant tumors anatomically localized in nerve trunks.In m a t instances, this soft tissue involvement occurs in, or in close association with, a conglomerate mass of proliferating mesenchymal tissues and plexiform neuromas.When a malignant tumor develops in such a conglomerate mass, it is difficult to determine the locus of origin, inasmuch as the tumor has violated all structural barriers within the area.The formation of tissues not ordinarily associated with nerve is not necessarily indicative of extraneural origin, since occasional cases of malignant metaplasia have