Zucca Emanuele, Gregorini Anna, Cavalli Franco
Research Division, Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona.
Ther Umsch. 2010 Oct;67(10):517-25. doi: 10.1024/0040-5930/a000088.
Primary extranodal lymphomas are relatively rare non-Hodgkin lymphoma presentations with either no or only "minor" nodal involvement along with a clinically "dominant" extranodal component, to which primary treatment must often be directed. Clinical presentations depend largely on the localization and are similar to those of other malignancies affecting that specific organ. In addition to the histological subtype, the primary organ of origin represents the most significant prognostic factor due to differences in natural history and, mainly, in management strategies related to organ-specific problems. In principle, as for primary nodal disease, treatment strategies depends on the patient's clinical conditions, the extent and/or location of the disease, and the histological type. In general, for stage I and II disease with low tumor burden, local therapy is a relevant option both for cure and local control. In advanced stage disease, systemic chemoimmunotherapy is usually required. Localizations with particularly poor survival are the enteropathy-type T-cell lymphoma, the primary testicular diffuse large B-cell lymphoma, and the primary CNS Lymphoma. However, recent studies have shown that site-tailored treatment strategies in testis and brain lymphoma may result in a significant outcome improvement. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is the most common indolent subtype. This lymphoma usually arises in mucosal sites where lymphocytes are not normally present and where a lymphoid infiltration is acquired in response to either chronic infectious conditions or autoimmune processes: Helicobacter pylori gastritis, Hashimoto's thyroiditis, Sjögren syndrome. Indeed, a pathogenetic link between gastric MALT lymphoma and H. pylori is strongly suggested by the regression of gastric MALT lymphoma (in approx. 75% of cases) after antibiotic eradication of the microorganism.
原发性结外淋巴瘤是相对罕见的非霍奇金淋巴瘤表现形式，其要么无淋巴结受累，要么仅有“轻微”淋巴结受累，同时伴有临床上“占主导”的结外成分，而这通常是主要治疗的靶向部位。临床表现很大程度上取决于病变部位，与影响该特定器官的其他恶性肿瘤相似。除了组织学亚型外，由于自然病程不同，主要是与器官特异性问题相关的管理策略不同，原发器官是最重要的预后因素。原则上，与原发性淋巴结疾病一样，治疗策略取决于患者的临床状况、疾病的范围和/或部位以及组织学类型。一般来说，对于肿瘤负荷低的Ⅰ期和Ⅱ期疾病，局部治疗是治愈和局部控制的相关选择。在晚期疾病中，通常需要进行全身化学免疫治疗。生存率特别低的部位是肠病型T细胞淋巴瘤、原发性睾丸弥漫性大B细胞淋巴瘤和原发性中枢神经系统淋巴瘤。然而，最近的研究表明，针对睾丸和脑淋巴瘤的针对性治疗策略可能会显著改善预后。黏膜相关淋巴组织结外边缘区淋巴瘤（MALT淋巴瘤）是最常见的惰性亚型。这种淋巴瘤通常发生在正常情况下不存在淋巴细胞的黏膜部位，在这些部位，由于慢性感染或自身免疫过程，会出现淋巴细胞浸润：幽门螺杆菌胃炎、桥本甲状腺炎、干燥综合征。事实上，抗生素根除幽门螺杆菌后，胃MALT淋巴瘤消退（约75%的病例），强烈提示胃MALT淋巴瘤与幽门螺杆菌之间存在致病联系。
