摘要目的 探讨儿童青少年黏膜相关淋巴组织结外边缘区(MALT)淋巴瘤的病理特征及临床表现.方法 观察5例儿童青少年MALT淋巴瘤镜下形态及免疫表型,并进行临床随访.结果(1)5例MALT淋巴瘤分别发生在胃窦、眼眶、腮腺及鼻咽部,患者均无B症状,仅表现为局部肿块和溃疡,其中1例伴有颈部淋巴结肿大,余4例均无淋巴结肿大.(2)5例均具有成人MALT淋巴瘤的病理特征,细胞形态表现为单核样B细胞或中心细胞样细胞,伴有浆细胞分化(2/5),可见滤泡植入及淋巴上皮病变(3/5).(3)免疫表型没有特异性的标志物,需综合诊断.(4)2例免疫球蛋白(Ig)基因重排检测阳性.(5)4例得到随访,1例予利妥昔单抗治疗,1例服用抗幽门螺杆菌药物治疗,2例未进一步治疗.4例均生存,且未见复发.结论 儿童青少年MALT淋巴瘤具有成人MALT淋巴瘤相似的病理特征及惰性的临床经过,预后较好.但由于患者发病年龄低,常容易误诊及漏诊.

abstractsObjective To investigate the pathological features and clinical manifestations of mucosa-associated lymphoid tissue (MALT) lymphoma in children and adolescents.Methods Five cases of MALT lymphoma in children were investigated by morphology and immunophenotyping along with clinical follow-up.Results Five cases of MALT lymphoma occurred in the antrum, orbit, parotid gland and nasopharynx.All patients had no B symptoms and only one patient showed a local mass with ulcer.One case presented with cervical lymph node enlargement, and 4 cases showed no evidence of lymphadenopathy.All cases had pathological features similar to those of adult MALT lymphoma, with proliferation of monocytoid B cells orcentralcyte-like tumor cells,with plasma cell differentiation and lymphoid epithelial lesions.No specific immunophenotypic marker was found.Clonal Ig gene rearrangement was detected in two cases.One patient was treated with rituximab treatment, 1 patient was given anti-Helicobacter pylori therapy, and 2 patients had no additional treatment.The follow-up data showed that 4 patients survived without tumor recurrence.Conclusions Similar to adult patients, MALT lymphoma in children and adolescents has similar pathological features with indolent clinical course and good prognosis.It is important to note that misdiagnosis and incorrect diagnosis mightoccur because of the young age of the patients.