ｖｏｎ　Ｒｅｃｋｌｉｎｇｈａｕｓｅｎ病に伴う腸間膜原発悪性末梢神経鞘腫の１例

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  • Mesenteric malignant peripheral nerve sheath tumor in a patient with von Recklinghausen's disease. Report of a case.

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We report a case of malignant peripheral nerve sheath tumor (MPNST) of the mesenterium in a patient with von Recklinghausen's disease (VRD).<br> Case: A 28-year-old woman•was admitted with abdominal fullness and right abdominal pain. Abdominal CT scan showed a 8×7cm in diameter irregularly enhanced tumor in the abdominal cavity, and a 3cm in diameter tumor in the left lateral segment of the liver. Angiography showed that the tumor was not well vascularised. The tumorectomy was performed by partial transverse colectomy and left lateral segmentectomy of the liver. Histological examination confirmed the diagnosis of MPNST and metastatic liver tumor. She died of the abdominal dissemination and the liver metastasis 3 months after the operation.

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