title: Malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis: a case report and a literature review.
abstract: A case report of a 56-year-old man with the characteristic clinical and histologic features of multiple neurofibromatosis is presented. This patient was also diagnosed as having a malignant fibrous histiocytoma involving the right thigh with metastasis to the lung. A definite association between Von Recklinghausen's disease and various sarcomas (most commonly neurofibrosarcomas) of the peripheral nerves and the somatic soft tissues is well-known. This is apparently the first report in the literature of a malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis. The phenomenon of sarcomatous change in Von Recklinghausen's disease and the clinical and pathologic features of malignant fibrous histiocytoma are also discussed.
mesh_terms: D018219:Histiocytoma, Benign Fibrous / Q000150:complications* / Q000473:pathology / Q000556:secondary; D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology / Q000556:secondary; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009456:Neurofibromatosis 1 / Q000150:complications*; D013848:Thigh