Abstract
von Recklinghausen病（以下，VR病と略記）に十二指腸gastrointestinal stromal tumor（以下，GISTと略記），十二指腸乳頭神経内分泌腫瘍，神経鞘腫を合併した1例を経験した．症例は45歳の男性で，黒色便を認め受診した．上部消化管内視鏡検査で十二指腸水平脚に粘膜下腫瘍を認めた．造影CTで十二指腸水平脚28 mm大，十二指腸乳頭8 mm大，胆囊頸部35 mm大の腫瘤を認めた．以上の所見から，VR病に伴う十二指腸多発GIST，胆囊神経鞘腫と診断し幽門輪温存膵頭十二指腸切除術を行った．病理診断は十二指腸水平脚腫瘍はGIST，十二指腸乳頭腫瘍は神経内分泌腫瘍，胆囊頸部腫瘍は神経鞘腫であった．術後補助療法は行わず19か月無再発生存中である．VR病で併存疾患を検討する際は，組織学的に異なる起源の腫瘍が同時期に発生する可能性を考慮し全消化管の検索が重要である．
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