von Recklinghausen病に合併した皮膚神経線維肉腫の1例

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  • A CASE REPORT OF NEUROFIBROSARCOMA OF THE SKIN WITH VON RECKLINGHAUSEN'S DISEASE

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A 64-year-old female had been having many masses at the whole body from infant age, and was admitted to the hospital because one of these masses on the left upper abdomen started to enlarge and bleed. On admission, we diagnosed her as von Recklinghausen's disease for body findings and family history. The mass measuring 6×5×3cm was smooth in surface and elastic soft. From these findings, a malignant tumor was suspected and the tumor was extirpated. Histopathological diagnosis was neurofibrosarcoma. Though we examined the whole body after the operation, no other lesions existed. Some cases of neurofibrosaroma with von Recklinghausen's disease have been reported. It is suspected that the neurofibrosarcoma occurred because of the malignant change of neurofibroma of the skin previously existed in our case. Such malignant change of neurofibroma seen in this case occurs uncommonly. It has been believed that neurofibrosarcoma of skin with von Recklinghausen's disease metastasizes or recurs in only a few cases, but tumor resection with a wide surgical margin is the treatment of first choice and careful postoperative follow-up would be mandatory.

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