Vater乳頭部癌を伴い複雑な病態を呈したRecklinghausen病の1例

von Recklinghausen's Disease with Adenocarcinoma of the Papilla Vater, Report of a Case

　Recklinghausen病（以下R病と略す）は，皮膚の色素斑（café au lait spots）と多発性神経線維腫を主徴とし，更に中枢神経系腫瘍の発生や眼病変，骨変化などを伴う遺伝性疾患である．最近，R病に非神経原性悪性腫瘍の合併する頻度が，従来言われていたより高いとの報告がみられる．

　われわれは，52歳，女性のR病にVater乳頭部癌を合併し，かつ，十二指腸漿膜側にも平滑筋腫を伴うといった多彩な病態を呈した1例を経験したので報告する．

　We reported A 51 year-old woman with von Recklinghausen's disease associated with adenocarcinoma of the papilla vater and leiomyoma in the serosa of the duodenum.

　von Recklinghausen's disease is a disease chracterized by dominant inheritance, caé au lait spots and neurofibromatosis. Moreover, it may be accompanied by deformity of the bone, eye disease, and tumor of the central nervous system. Recently it is reported that the incidence of occurrence of the nonneurogenic malignant tumor is more than just low. There are only two reported cases of those with von Recklinghausen's disease accompanied by adenocarcinoma of the papilla Vater which include the one just reported. The possibility of not only malignant change of nurofibroma but that of non-neurogenic malignant tumor should be kept in mind in the examination of von Recklinghausen's disease.