A case of repeated gastrointestinal bleeding from a malignant peripheral nerve sheath tumor of gastrointestinal tract

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  • 消化管出血を繰り返した小腸と胃の悪性末梢神経鞘腫の1例
  • 症例 消化管出血を繰り返した小腸と胃の悪性末梢神経鞘腫の1例
  • ショウレイ ショウカカン シュッケツ オ クリカエシタ ショウチョウ ト イ ノ アクセイ マッショウ シンケイ サヤ シュ ノ 1レイ

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Abstract

In May 2009, a 59-year-old woman with neurofibromatosis type 1 was operated for masses of the head and left shoulder growing to 10 cm in diameter over 6 months, and the pathological diagnosis was malignant peripheral nerve sheath tumors (MPNST). In April 2010, she developed anemia. Computed tomography (CT) and positron emission tomography (PET) showed a mass of in both the intestine and the left lung. A 1 cm diameter gastric mass was also shown, but the biopsy was negative and there was no bleeding. Suspecting bleeding from the intestinal mass, a partial resection of the small intestine was performed and the mass was diagnosed as MPNST. After 2 weeks from discharge, she developed anemia again, and gastric mass showed to be growing and bleeding. Partial gastrectomy was performed and after 4 weeks, a left lingular segmentectomy was performed. Both were diagnosed as MPNST. After 4 weeks, she developed pulmonary embolism and multiple masses in the intestine, peritoneum, subcutaneous tissue. She developed small-intestinal intussusceptions, obstructive ileus and bleeding, so a partial resection of the small intestine was performed and diagnosed as MPNST. <BR>The next day she was discharged, she developed disturbance of consciousness and left-side partial paralysis and was diagnosed with multiple brain metastases, and has subsequently died. <BR>MPNSTs are rare tumor entities that originate from peripheral nerve sheaths. Gastrointestinal MPNSTs have been reported in only 15 cases, including ours, in Japan.

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