[Malignant schwannoma arising in Recklinghausen's disease--report of 4 cases].

Four cases of malignant schwannoma arising in Recklinghausen's disease are described. The 4 patients, aged 38, 41, 44 and 38, 2 men and 2 women, had up to child-head-sized tumors in the neck, back, axilla and retroperitoneum. Oncostatic chemotherapy and irradiation were ineffective against the malignant schwannoma. In three, surgically tumor-resected cases, local recurrence and rapid tumor growth occurred. Histologically, malignant schwannoma was characterized by the presence of a few collagen fibers among the tumor cells, but abundant argentaffin fibers. Numerous mast cells are frequently seen in neurofibroma, but almost never in malignant schwannoma. In Case 3, with mild atypism, tumor cells were positive for S100 protein.