Turcot症候群の1例

Turcot Syndrome, Report of a Case

要旨　患者は19歳，男性．小脳腫瘍（髄芽腫）手術および術後放射線療法後に鉄欠乏性貧血を認め，当科に紹介された．下部消化管内視鏡検査で大腸ポリポーシスを認め，脳腫瘍との合併からTurcot症候群と診断した．上部消化管内視鏡検査では，胃底腺ポリポーシスと，多発性十二指腸病変を認めた．髄芽腫に対する化学療法終了後に十二指腸部分切除術が施行され，十二指腸水平部の広基性隆起性病変は腺腫内癌と診断された．本邦では，1983年以降Turcot症候群として26例が報告されている．脳腫瘍より消化管癌が先行する症例が少なからず存在するため，若年者の大腸ポリポーシスを認めた際には，本疾患を念頭に置き診療に当たる必要があると考えられた．

　A 19-year old man was referred to our department because of iron deficiency anemia. He had been treated in the neurosurgery department of our hospital for glioblastoma since 4 months previously. He had an operation for glioblastoma, then he had postoperative radiation therapy. Esophagogastroendoscopy showed multiple flat elevated lesions with depressions and a large lesion resembling a LST（laterally spreading tumor）measuring about 40mm in the duodenum. It also showed fundic gland polyposis in the upper and the middle portion of the stomach. The duodenal lesions were diagnosed histopathologically as having low grade atypia. Total colonoscopy showed polyposis coli and colorectal polyps were diagnosed as tubular adenoma with low grade atypia.

　Physical findings and CT examination indicated no lesions on his skin, thyroid, bone, etc. Based on those findings, he was diagnosed as having Turcot syndrome. After chemotherapy for glioblastoma, partial duodenectomy was performed for the LST-like duodenal lesion. It contained well differentiated type tubular adenocarcinoma in the lamina propria mucosae. In sequential treatment, he had complete clinical remission and has continued under ambulatory medical care. Iron deficiency anemia also improved after partial duodenectomy. Twenty six cases of Turcot syndrome have been reported in Japan since 1983 and their clinical features were reviewed.