Neurogenic sarcomas in patients with neurofibromatosis (von Recklinghausen's disease)

Abstract

Thirteen soft tissue neurogenic sarcomas from twelve patients with neurofibromatosis (Von Recklinghausen's disease) were ultrastructurally examined. Electron microscopic studies revealed a wide spectrum of morphological manifestations varying from schwannian to fibroblastic, histiocytic, fibrohistiocytic and relatively undifferentiated cellular proliferations. A similar variation on light microscopic appearances has been previously reported in these neurogenic sarcomas. Neurogenic sarcomas occurring in patients with neurofibromatosis (Von Recklinghausen's disease), represent a heterogenous group of neoplasms with various patterns of differentiation identified ultrastructurally. The morphologic expressions of these neurogenic neoplasms can be conceptualized as a disorderly growth of the various peripheral nerve cellular components, or, as has been previously suggested, as a result of the multipotential nature and metaplastic ability of Schwann cells. S-100 protein immunohistochemistry was only positive in those neoplasms ultrastructurally proven to represent schwannian cellular proliferations. This study serves to document the range of fine structure that may be found in neurogenic sarcomas, to correlate the ultrastructural findings with the light microscopic appearance of these tumors, to determine the specificity of the electron microscopic findings, and immunohistochemistry for S-100 protein and assess their possible value in differential diagnosis.