胃肠道原发性间变性大细胞淋巴瘤临床病理分析

Clinicopathological analysis of primary gastrointestinal anaplastic large cell lymphoma

目的探讨胃肠道原发性间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的病理形态、免疫表型特征及临床预后。方法对5例胃肠道原发ALCL进行形态观察,免疫组化标记及随访,并结合相关文献进行讨论。结果本组ALCL男女之比为1.5∶1,平均年龄40.6岁。发生部位包括胃2例,胰、十二指肠1例,回盲部1例,结肠1例。所有病例均经外科手术切除,2例术后进行化疗。5例中有4例随访3至12个月,其中1例死亡,3例健在。ALCL形态表现多样,细胞呈多形性,大小不等,胞质丰富,细胞核大而不规则,呈扭曲、肾形、马蹄形及花环状,甚至见多核及巨核瘤细胞。免疫表型:5例ALCL均阳性表达CD30,部分表达EMA、CD3和CD45RO,而不表达CKpan、CD20、HMB45、CD68、CD15和CD117。结论 ALCL是一种少见的非霍奇金淋巴瘤,发生在胃肠道罕见,有必要与霍奇金淋巴瘤、弥漫性大B细胞淋巴瘤、低分化腺癌、胃肠道上皮样间质瘤、恶性纤维组织细胞瘤等进行鉴别。

Purpose To explore the histological,immunohistochemical characters and prognosis of primary gastrointestinal anaplastic large cell lymphoma（ALCL）.Methods Histology,immunohistochemical markers and follow-up information of five primary gastrointestinal ALCL cases were analyzed and the correlated literature was reviewed.Results The ALCL of five patients had 1.5 ∶1 male/female ratio with an average age of 40.6（range,22~62 years）.2 cases were located in the stomach,1 in pancreas and duodenum,1 in ileocecal junction,1 in colon.All patients underwent operation and two patients received chemotherapy after surgery.Four patients were subjected to follow-up of 3 to 12 months,one died and three survived.ALCL showed distinctive morphological characters.The tumors were mainly composed of large and pleomorphic cells with abundant cytoplasm.The nuclei were large and irregular with shape of twist,horseshoed and wreathlike.Multiple nuclei or huge nuclei were seen sometimes.All five cases expressed CD30 and partly expressed CD3,EMA and CD45RO.All five cases were negative for CKpan,CD20,HMB45,CD68,CD15 and CD117.Conclusions Primary gastrointestinal ALCL is extremely rare.It is necessary to differentiate it from other type of tumors such as the diffuse large B cell lymphoma,Hodgkin lymphoma,poor differentiated carcinomas,epithelioil gastrointestinal stromal tumors and malignant fibrous histiocytoma.