Neurofibrosarcoma of the radial nerve in von Recklinghausen's disease with metastatic angiosarcoma.
A case of von Recklinghausen's disease with multiple neurofibromatosis is described in which death was caused by intracerebral haemorrhage due to metastatic angiosarcoma. The primary site was a peripheral nerve tumour which, on microscopic examination, showed both angiosarcoma and neurofibrosarcoma with transitional zones between the two. This is interpreted as another example of divergent differentiation in malignant nerve sheath tumours, and the case is compared with the two similar examples found in the literature.
D000293:Adolescent; D001932:Brain Neoplasms / Q000150:complications*; D006394:Hemangiosarcoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology; D010524:Peripheral Nervous System Neoplasms / Q000150:complications* / Q000473:pathology