von Recklinghausen病に合併した十二指腸悪性神経鞘腫の1例

Malignant Schwannoma of the Duodenum and Various Nerve Proliferative Lesions of the Intestinal Tract in the Patient with Neurofibromatosis

要旨　患者は45歳のvon Recklinghausen病（R病）の女性，貧血症状を主訴に受診し，消化管の臨床的および病理学的検索により十二指腸の悪性Schwann鞘腫をはじめ十二指腸，空腸，大腸に神経線維腫，末梢神経組織の過形成など多彩な消化管の病変を認めた．現在までR病に消化管病変が合併する頻度は剖検例で比較的高いと言われてきたが，本例のような一連の合併病変を有した症例の報告はない．更に本例は術前に詳細な検索によりその形態学的特徴を捉えられた．以上の点で貴重な症例と考えられた．

　A 45-year-old woman with von Recklinghausen neurofibromatosis developed a malignant Schwannoma with two neurofibromas of the duodenum, and small elevated lesions (hyperplasia of the peripheral nerve) of the small intestine and colon.

　Although gastrointestinal neurogenic tumor has been known to occur frequently in patients with von Recklinghausen neurofibromatosis, such a case as described here has not yet been reported. Results of clinical investigation in this case were reported as well as radiological, endoscopic and pathological features of the lesions in detail.