胃・空腸腫瘍を伴ったレックリングハウゼン病の1症例

Schwannoma of the Stomach and Jejunum in von Recklighausen Disease, a Case Report

　レックリングハ・ウゼン病（neurofibromatosis，以下レ病と略す）は，1882年von Reckling hausenにょって初めて報告された，café au lait spotsと，多発せる皮膚腫瘤を主徴とする疾患である．出生約3,000人に対し1人の頻度で発生し，濃厚な遺伝関係を有するとされ，最近では，系統的疾患として，骨の変化，中枢神経系腫瘍の合併，消化管腫瘍の合併，副腎褐色細胞腫の合併などが，腫瘍の悪性化の問題とともに注目されている．われわれは，特有な骨変化を有し，胃・空腸腫瘍を伴ったレ病の一症例を経験したので報告する．

　A case of von Recklinghausen disease associated with Schwannoma of the stomach and jejunum is described. Von Recklinghausen disease characterized by café au fait spots and multiple neurofibromas of the skin is regarded as a systemic disease combining bony abnormalities, neoplasms of the central nervous system, gastro-intestinal neoplasm, and pheochromocytoma. Also malignant changes is reported in 2～16%.

　The patient, a 74-year-old man, had a discomfort upon the upper abdomen. Upper G-Ⅰ series and gastrofiberscopic examination revealed a submucosal tumor of the greater curvature in the antrum. Bone and joint x-ray indicated the right scoliosis and the defect of the sphenoidal bone. Blood-chemical examination, plain films of the chest and abdomen, intravenous pyelogram, and barium enema showed no abnormal findings.

　By laparotomy two subserosal tumors of the jejunum were found in addition to the gastric tumor. Gastrectomy by Billroth Ⅱ method, extirpation of the jejunal tumor and a part of the skin containing cutaneous tumor were performed.

　Pathological diagnosis was proved as malignant schwannoma of the stomach and jejunum, and neurofibroma of the skin. Jejunal tumor was considered as less malignant than that of the stomach, and contained smooth muscle component.

　The G-Ⅰ tract is rarely involved in the patient having neurofibromatosis. In the case of neurogenic neoplasm subserosal nerve is the usual origin and intramuscular and submucosal nerve is uncommon. In general neurofibroma and Schwannoma are considered to originate in Schwann cell of the nerve sheath, but the difference of the histological picture between them is still a much debated point.

　Although most of those who have neurogenic neoplasm in G-I tract are symptomless, they are not infrequently accompanied by adbominal pain, anemia, G-Ⅰ bleeding, intestinal obstruction, perforation, or intussusception. Considering the malignant change, surgical resection in early stage is the most recommended therapy.