レックリングハウゼン病に合併し肝転移をきたした巨大胃神経肉腫の1例

Von-Recklinghausen's Disease Associated with Neurofibroma of the Stomach, a Case Report

　レックリングハウゼン病(以下レ病)に伴なう内臓病変，ことに消化管病変についてはGrodsky ＆ Francisco¹⁾，Williams ＆ Pollack²⁾等何例かの症例に接し，Palmer³⁾も同症に生じた28例の胃神経鞘腫例を蒐めているが，比較的稀な症例である．レ病は1882年von Recklinghausenにより皮膚のcafé au lait spotと皮下神経線維腫の多発を主徴とする疾病として記載されたが，メンデル優性型遺伝を示し，屡々内臓の神経系の異形性，時に内分泌腺の腺腫形成を伴なうことから先天性(遺伝性)全身性母斑症として理解されるようになった．Stout⁴⁾は更にSturge-Weber病，Hipple-Lindeau病も一括し，その中に中枢神経型，末梢神経系型，内臓神経線維腫症型及び少数の特殊型の四亜型を設けて論じている．

　最近定型的な経過を示したレ病の胃に巨大なポリープ状の神経肉腫を形成し，切除後広般な肝転移を来して死亡した興味ある1例を経験した．Stout⁴⁾によれば内臓神経線維腫症に含まれるべき病像を示しており，その悪性化，併存した膵β細胞腫等なお検討の余地を残しているが，胃病変を中心に報告したい．

　A 69 years old farmer manifested typical clinical history of von Recklinghausen's disease with many café-au-lait spots, dermal neurofibromatous tumors, retarded inteligence, paresis of left trigeminus nerve, etc.

　The patient was admitted to our Hospital in April 1973 because of gastric tumor associated with continued melena and occasional hematemesis. Roentogenologically a large irregularly shaped movable polypoid lesion was detected on the relatively upper portion of the stomach. During the examination and treatment in our Hospital, the patient suddenly appeared extrahepatic jaundice and then pressing operation was done to resect the large gastric tumor. A large myxoid and polypoid lesion had developed on the cardiac mucosa and extended to the second portion of the duodenum through the pyloric ring pressing upon the Vater's pailla by edematously swollen and hemorrhagic head of the lesion. The lesion was completely resected together with basic gastric wall but the patient died after 3 weeks by marked liver metastases and decreased general condition. Microscopic examination revealed that many neurofibromas and dysplastic conditions of peripheral nerve developing on the gastrointestinal canal and some malignant lesions developed on them in the stomach as described above and the jejunum. Anaplastic spindle-shaped or satellite-type sarcoma cells loosely developing with rich myxoid stroma and basement membrane associatedly developed with these malignant cells on electrono-microscopic appearance suggest these cells as nervous origin. Associatedly insuloma was also observed ectopically at the duodenal wall and clinically hypoglycemia was suspected.