A case of schwannosarcoma associated with a pleural neurofibroma and a parathyroid adenoma is presented. The neuroectodermal origin and the relationship with neurofibromatosis are discussed. The sarcomatous relapse of a schwannoma previously classified as benign confirms the difficulty of microscopic analysis and the malignancy power of the tumor. A long survival is obtained by a large intestinal and mesenteric resection as long as hepatic metastases are not present or can be resected. The pleural neurofibroma needs symptomatic treatment while parathyroid adenoma must be early removed as soon as malignant hypercalcemia is detected.
