Steinbrecher Oskar, Kiesewetter Barbara, Dolak Werner, Brand Rosa, Simonitsch-Klupp Ingrid, Raderer Markus
Division of Oncology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Hematol Oncol. 2025 Jan;43(1):e70007. doi: 10.1002/hon.70007.
Extranodal marginal zone B-cell lymphoma of the mucosa associated lymphoid tissue (MALT-lymphoma) is an indolent B-cell lymphoma with a distinct affinity for mucosal structures. Most commonly arising in the stomach, only roughly 2% of MALT-lymphomas occur in the colon or the intestine. In view of this, we have retrospectively assessed all patients with MALT-lymphoma involving the intestine for clinicopathological characteristics. Data of all patients with MALT-lymphoma and intestinal involvement (i.e. both primary and secondary), treated and followed at the Medical University of Vienna between 1999 and 2021 were retrospectively collected from hospital records and analyzed. Differences in baseline and therapy characteristics, as well as survival between primary and secondary, and between intestinal and gastric MALT-lymphoma (as the most common subgroup of patients) were investigated. In total, 42 patients were identified; 24/484 (5%) were classified as primary and 18 (3.7%) as secondary intestinal MALT-lymphomas. The most common primary intestinal location was the colon (10/24) and the most frequent primary site in the 18 cases with secondary intestinal MALT-lymphomas was the stomach (14/18). A total of 28/42 (66.7%) patients presented with LUGANO stage I, 7/42 (16.7%) with stage II/IIE and 7/42 (16.7%) with stage IV disease. Translocation t (11; 18) (q21; q21) was positive in 47% of patients with secondary and 25% of primary intestinal MALT-lymphomas. Median OS in for intestinal MALT-lymphoma was 301 months (95% CI n.a.) with 89.1% alive at 5 years and 77.2% alive at 10 years. Median PFS in the entire cohort was 50.4 months (95% CI 38.4-62.4 months), with an overall response rate and disease control rate of 73% and 97.3%, respectively. No difference in OS and PFS between primary and secondary intestinal, as well as between intestinal and gastric MALT-lymphoma was detected. Our data suggest that dissemination within the GI tract does not seem to be an adverse prognostic feature and highlights the preferred use of the Lugano staging system in such patients, which also summarizes multiple lesions within the GI-tract as Stage I.
黏膜相关淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）是一种惰性B细胞淋巴瘤，对黏膜结构具有独特的亲和力。MALT淋巴瘤最常发生于胃部，仅有约2%发生于结肠或小肠。鉴于此，我们回顾性评估了所有肠道受累的MALT淋巴瘤患者的临床病理特征。回顾性收集了1999年至2021年间在维也纳医科大学接受治疗和随访的所有肠道受累（包括原发性和继发性）的MALT淋巴瘤患者的数据，并进行分析。研究了原发性和继发性肠道MALT淋巴瘤以及肠道和胃部MALT淋巴瘤（作为最常见的患者亚组）在基线和治疗特征以及生存率方面的差异。总共确定了42例患者；24/484（5%）被分类为原发性肠道MALT淋巴瘤，18例（3.7%）为继发性肠道MALT淋巴瘤。原发性肠道最常见的部位是结肠（10/24），18例继发性肠道MALT淋巴瘤中最常见的原发部位是胃（14/18）。42例患者中共有28例（66.7%）为LUGANO I期，7例（16.7%）为II/IIE期，7例（16.7%）为IV期。t（11；18）（q21；q21）易位在47%的继发性肠道MALT淋巴瘤患者和25%的原发性肠道MALT淋巴瘤患者中呈阳性。肠道MALT淋巴瘤的中位总生存期为301个月（95%CI未提供），5年生存率为89.1%，10年生存率为77.2%。整个队列的中位无进展生存期为50.4个月（95%CI 38.4 - 62.4个月），总缓解率和疾病控制率分别为73%和97.3%。未检测到原发性和继发性肠道MALT淋巴瘤以及肠道和胃部MALT淋巴瘤在总生存期和无进展生存期方面的差异。我们的数据表明，胃肠道内的播散似乎不是一个不良预后特征，并强调了在这类患者中优先使用LUGANO分期系统，该系统也将胃肠道内的多个病变总结为I期。
