PRIMARY JEJUNAL MALIGNANT MIXED TUMOR IN A PATIENT WITH VONRECKLINGHAUSEN NEUROFIBROMATOSIS

A rare case of primary jejunal malignant mixed tumor arising in a 49-y r-old Japanese male with von Recklinghausen's disease is reported. The patient, who had a past history of partial gastrectomy due to duodena l ulcer, was admitted with a complaint of epigastric pain. Upper gastr ointestinal examinations showed a huge polypoid tumor located in the e fferent loop of the gastrojejunostomy site. Because the tumor was stro ngly suggestive of leiomyosarcoma on histological examination of biops y specimens, laparotomy was performed. The resected tumor measuring 10 x 7 x 7 cm was composed of adenocarcinoma admired with various sarcom atous components, including rhabdomyosarcoma, osteosarcoma, and other sarcomas. Immunohistochemical analysis also supported this diagnosis. The features of this tumor closely resembled malignant mixed mullerian tumor of heterologous type that develops in female genital organs. It is well known that patients with von Recklinghausen neurofibromatosis have an increased incidence of mesenchymal tumors and malignant neopl asias, and therefore, it seems that there is a possible relationship b etween the histogenesis of this peculiar tumor and the genetic abnorma lity in this patient.