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We reported a case of von Recklinghausen's disease complicated by macroglobulinemia, malignant Schwannoma and pheochromocytoma. A 43-year-old male patient who was diagnosed as von Recklinghausen's disease developed abdominal masses at 5 years after the adrenalectomy for pheochromocytoma. Computed tomography showed multiple heterogeneous tumors in abdominal cavity. Histological examination of resected tumor was compatible with malignant Schwannoma. At the same time, peripheral blood and bone marrow smears showed a large number of plasmacytoid lymphocytes. Immunoelectrophoresis revealed M-protein of IgM, kappa type. He was also diagnosed of coexistence with macroglobulinemia. The chemotherapeutic protocols used were not effective against both malignant Schwannoma and macroglobulinemia. The present case is a rare case of von Recklinghausen's disease associated with triple neoplasms.
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