Abstract
We report a 51-year-old Japanese man with malignant schwannoma associated with von Recklinghausen’s disease. The dome-shaped tumor developed on the right side of his chest and was elastic hard, purplish brown with a diameter of 8 cm. Histopathology showed the nodule composed of strongly deformed cells that were spindle or oval shaped while displaying a storiform pattern. Mitosis and bizarre cells, usually observed in malignant fibrous histiocytomas, were present, but the immunoreactivities of S-100 protein and neuron specific enolase and the presence of basal lamina on an electron microscopic examination led to the diagnosis of malignant schwannoma. The patient was treated by a surgical wide excision and postoperative chemotherapy.
