空・回腸悪性リンパ腫168例の臨床病理学的特徴―X線・内視鏡所見を中心に

Primary Jejunoileal Lymphoma: A Clinicopathological Analysis of 168 Patients

要旨　空・回腸悪性リンパ腫168例〔MALTリンパ腫20例，濾胞性リンパ腫40例，マントル細胞リンパ腫5例，DLBCL（diffuse large B-cell lymphoma）69例，Burkittリンパ腫7例，リンパ芽球性リンパ腫2例，NK/T細胞リンパ腫25例〕の臨床病理学的特徴を遡及的に解析した．DLBCL・MALTリンパ腫は回腸，濾胞性リンパ腫は空腸に好発し，NK/T細胞リンパ腫やマントル細胞リンパ腫は小腸の広範囲に病変を来す傾向がみられた．肉眼型は，隆起型39例，潰瘍型60例，MLP（multiple lymphomatous polyposis）型38例，びまん型14例，混合型17例であり，潰瘍型・隆起型はDLBCL，MLP型は濾胞性リンパ腫，びまん型はNK/T細胞リンパ腫で多くみられた．診断5年後のoverall survivalおよびprogression-free survivalは59％および50％であり，免疫表現型，組織型（非indolent），治癒切除の有無が両者に共通した予後規定因子であった．空・回腸リンパ腫の診断には，各組織型に特徴的な肉眼所見を理解して，適切な画像検査を行うことが重要である．

　The clinicopathological features of 168 patients with primary jejunoileal lymphoma〔20 cases of MALT lymphoma, 40 follicular lymphoma, 5 mantle cell lymphoma, 69 DLBCL（diffuse large B-cell lymphoma）, 7 Burkitt lymphoma, 2 lymphoblastic lymphoma, 25 NK/T-cell lymphomas〕were retrospectively investigated. While DLBCL and MALT lymphoma frequently involved the ileum and follicular lymphoma did the jejunum, NK/T-cell lymphomas tended to involve extensive areas of the small bowel. Macroscopically, these cases were classified either as polypoid（n＝39）, ulcerative（n＝60）, polyposis（n＝38）, diffuse（n＝14）or combined（n＝17）forms. The ulcerative and polypoid forms were predominant in cases of DLBCL and MALT lymphoma, polyposis form in follicular lymphoma, and diffuse type in NK/T-cell lymphomas. Overall and progression-free survival rates at 5 years were 59％ and 50％, respectively. Cox multivariate analysis revealed that the immunophenotype, histologic type, and radical surgery were independent prognostic factors. For the accurate clinical diagnosis of jejunoileal lymphoma, it is necessary to understand the characteristic macroscopic findings in each histologic type.