原发性淋巴结外间变性大细胞淋巴瘤临床病理分析

Clinicopathological features of primary extranodal anaplastic large cell lymphoma

目的探讨原发性淋巴结外间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的病理形态、免疫表型特征及预后特点。方法对29例原发性淋巴结外ALCL进行形态学观察、免疫组化标记及随访,并结合相关文献进行讨论。结果本组淋巴结外ALCL共29例,男女之比为1.6∶1,平均年龄46.2岁。发生于皮肤12例、消化道7例、骨5例、口鼻黏膜3例、肺及乳腺各1例。29例患者中随访12例,随访时间3～45个月。29例均经外科手术切除局部病变或脏器,大部分再辅以化疗和放疗。12例随访病例中,手术切除病变后接受单纯化疗7例,放化疗者2例,未经放化疗者3例。其中8例死亡。ALCL组织学形态多样。免疫组化:29例ALCL均表达CD30,大多数表达CD3和(或)CD43(26/29),部分表达EMA、ALK-1、GranB和Perforin;不表达CKpan、CD20、CD79α、HMB45、CD68、CD15和CD117。结论发生在淋巴结外的ALCL并非罕见,临床表现无特异性,诊断依赖于组织病理学及免疫组化标记,淋巴结外(除外皮肤)的ALCL预后相对较差。该病应与弥漫性大B细胞淋巴瘤、霍奇金淋巴瘤、低分化腺癌及胃肠道上皮样间质肿瘤等鉴别。

Purpose To explore the morphological, immunohistochemical characteristics and prognosis of primary extranodal anaplastic large cell lymphoma （ALCL）. Methods The histopathologic features, immunohistochemical markers and follow-up information were analyzed in 29 cases of primary extranodal ALCL and the correlated literature was reviewed. Results The ALCL of 29 patients had 1.6 ： 1 male/female ratio with an average age of 46. 2. 12 cases were located in the skin, 7 in the gut, 5 in the bone, 3 in the mucosa of oral and nose, 1 in the lung, and 1 in the breast. All patients underwent surgical removal and seven patients received chemotherapy after surgery, while 2 received chemotherapy and radiotherapy, and other 3 received nothing. Twelve patients were subjected to follow-up of 3 - 45 months, and 8 patients died. ALCL showed distinctive morphological characteristics. All 29 cases of ALCL expressed CD30 and 26 cases expressed CD3 and/or CD43. EMA, ALK-1, GranB and Perforin were positive in ALCL. All the ALCL were negative for CKpan, CD20, CD79a, HMB45, CD68, CD15 and CDl17. Conclusions Primary extranodal ALCL is not rare. The prognosis of ex- tranodal ALCL excluding skin ALCL is poor. It is necessary to differentiate it from other type of tumors such as the diffuse large B cell lymphoma, Hodgkin lymphoma, poor differentiated carcinomas and epithelioid gastrointestinal stromal tumors.