Malignant peripheral nerve sheath tumors arising from ganglioneuromas

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Abstract
Two typical malignant peripheral nerve sheath tumors (PNST) arising in preexisting ganglioneuromas are described. Neither patient had the stigmata nor family history of Von Recklinghausen''s neurofibromatosis. In both cases, the ganglioneuromas evolved from more primitive neuroectodermal tumors (1 neuroblastoma, 1 ganglioneuroblastoma) and both patients developed their malignant PNST at previously irradiated sites. Both patients died within 2 yr of the diagnosis of their malignant PNST. The origin of these malignant PNST from Schwann cells is supported by an ultrastructural analysis of 27 neuroblastomas, ganglioneuroblastomas and ganglioneuromas.