121例肠道原发性淋巴瘤临床病理分析

Clinicopathological analysis of primary intestinal lymphoma:a report of 121 cases

目的研究肠道原发性淋巴瘤的临床病理特点和预后。方法复习121例肠道原发性淋巴瘤临床资料、大体所见、HE切片、免疫组化染色,按2008年WHO造血与淋巴组织肿瘤分类重新进行分类,并用Kaplan-Meier法进行生存分析。结果121例肠道原发性淋巴瘤发病年龄从7～81岁,平均年龄52.7岁,中位年龄55岁。男性88例,女性33例,男女比例8∶3。发病部位分别为大肠(结、直肠)62例(51.2%),小肠(空、回肠)47例(38.8%),十二指肠7例(5.8%),多发部位4例(3.3%),阑尾1例(0.8%)。临床上以腹痛、腹胀(72.9%)、下消化道出血(22.0%)、发热(10.2%)、腹部包块(8.5%)、腹泻(8.5%)、消瘦(5.1%)等为首发症状。大体上表现为隆起型(37.3%)、溃疡型(33.9%)和浸润型(28.8%)。组织学类型分别为弥漫性大B细胞淋巴瘤,非特殊类型68例(56.2%),MALT淋巴瘤18例(14.9%),外周T细胞淋巴瘤,非特殊类型13例(10.7%),肠病相关T细胞淋巴瘤7例(5.8%),结外NK/T细胞淋巴瘤,鼻型4例(3.3%),套细胞淋巴瘤和滤泡性淋巴瘤各3例(2.5%),ALK阴性的间变性大细胞淋巴瘤2例(1.7%),慢性淋巴细胞性白血病/小淋巴细胞性淋巴瘤、Burkitt淋巴瘤和不能分类的B细胞淋巴瘤各1例(0.8%)。59例外检病例中共随访39例(66.1%),随访时间1-102月,平均随访时间19.7月,目前死亡15例。20例失访(未回信或拒绝告知)。结论肠道原发性淋巴瘤以中老年男性多见,最常累及的部位是大肠(结、直肠),临床表现以腹痛、腹胀多见;最常见的组织学类型是弥漫性大B细胞淋巴瘤,非特殊类型。肠道原发性T细胞淋巴瘤的预后比B细胞淋巴瘤差。

Objective To analyze the clinicopathological characteristics and prognosis of primary intestinal lymphoma. Methods The clinical and pathological data of 121 cases of primary intestinal lymphoma were reviewed. The pathology was examined by HE and immunohistochemical staining and reclassified according to the 2008 WHO classification of tumors derived from haematopoietic and lymphoid tissues. The clinical data were analyzed by Kaplan-Meier method. Results The age of patients ranged from 7 to 81 years,with an average of 52.7 years and median of 55 years. The male-to-female ratio was 8 ： 3（88 ： 33）. The most frequent site of the lesions was the large intestine （n=62,51.2%）,followed by small intestine （n= 47,38.8%）,duodenum （n= 7, 5.8%）,multiple intestinal sites （n=4, 3.3%） and appendix （n= 1,0.8%）. The clinical presentations included abdominal pain and/or distention （72.9%）; lower digestive tract hemorrhage （22.0%）, fever （10.2%）,abdominal mass （8.5%）, diarrhea （8.5%） and weight loss （5. 1%）. The major gross findings were bossing （37.3%）,ulcer （33.9%） and infiltrating （28.8%）. The diagnoses were： not specified diffuse large B-cell lymphoma （68/ 121,56.2% ） ,MALT Iymphoma （18/121,14.9%） ,not specified peripheral T-cell lymphoma （13/121,10.7%） ,enteropathy- associated T-cell lymphoma （7/121,5.8 % ）, extranodal NK/T cell lymphoma, nasal-type （4/121,3.3 % ）, mantle cell lymphoma （3/121,2.5%）,follicular lymphoma （3/121,2.5%）,anaplastic large ceil lymphoma,ALK-negative （2/121,1.7%）,chronic lymphocytic leukemia /small lymphocytic lymphoma （1/121,0.8%）,Burkitt lymphoma （1/121,0.8%） and unclassifiable B- cell lymphoma （1/121, 0.8%）. The follow-up time of 39 cases was 1-102 month with an average of 19.7 months. Conclusions Primary intestinal lymphoma can usually be found in mid-aged and elderly males. Large intestine （colon, rectum） is the most common site involved. Abdominal pain and/or distention are the common clinical manifestations. Diffuse large B-cell lymphoma ,not otherwise specified is the most common histological type. Compared with B-cell lymphoma ,primary intestinal T-cell lymphoma has a worse prognosis.