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KMID : 1188520140100020116
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Korean Journal of Clinical Oncology
2014 Volume.10 No. 2 p.116 ~ p.121
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Neurofibroma of the appendix and multiple gastrointestinal stromal tumors of small bowel in neurofibromatosis type 1 patient
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Jeong Gu-Ae
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Abstract
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Neurofibromatosis type 1 (NF-1) is an autosomal dominant hereditary disease with association of tumorous condition of body. Although the pathogenesis of tumorous condition with NF-1 is unclear, there were several reports for gastrointestinal tumors (GISTs) of gastrointestinal tract or nervous system tumors. However, there are few reports of appendiceal neurofibroma in NF-1 patients. We report here a case of NF-1 patient with multiple jejunal GISTs and appendiceal neurofibroma. A 61-year-old man was referred to department of surgery for abnormal findings of appendix on abdominal computed tomography scan. He underwent the laparoscopic appendectomy and laparoscopy-assisted segmental resection of proximal jejunum and he had numerous masses on duodenum and proximal jejunum in the operative findings. The pathologic findings revealed the neurofibroma of appendix and multiple GISTs of the jejunum.
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KEYWORD
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Neurofibromatoses, Jejunum, Gastrointestinal stromal tumors, Appendix, Neurofibroma
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