淋巴造血组织肿瘤构成的多原发肿瘤临床病理特征

Clinicopathological features of multiple primary malignant neoplasms constituted by tumors of hematopoietic and lymphoid tissues

目的 多原发恶性肿瘤（multiple primary malignant neoplasms,MPMNs）是指同一例患者同时或先后发生≥2个不同组织分化的原发恶性肿瘤。本研究旨在探讨淋巴造血组织肿瘤中MPMNs的临床病理特征,为临床诊疗提供依据。方法 对四川大学华西医院病理科2009-04-01-2014-01-31确诊的22例由淋巴造血组织肿瘤构成的MPMNs进行病理形态学、免疫表型及临床特征分析。结果 从7 898例淋巴造血组织肿瘤中筛选出22例（2.79‰）MPMNs患者。其中,同时性肿瘤20例,异时性肿瘤2例。第1肿瘤包括上皮性恶性肿瘤（实体癌18例）、髓系白血病（1例）、非霍奇金淋巴瘤（non-Hodgkin lymphoma,NHL）（2例）及高风险度胃肠间质瘤（1例）;第2肿瘤为NHL（18例）、经典型霍奇金淋巴瘤（1例）、高风险度胃肠间质瘤（1例）和实体癌（2例）。在22例由淋巴造血系统肿瘤构成的MPMNs中,B细胞和T细胞淋巴瘤分别为86.36%（19/22）和4.54%（1/22）;其中以慢性淋巴细胞性白血病/小淋巴细胞性淋巴瘤（chronic lymphocytic leukemia/small lymphocytic lymphoma,CLL/SLL）最为多见（36.36%）,其次为弥漫大B细胞淋巴瘤（diffuse large B cell lymphoma,DLBCL）27.27%。发生部位以胃肠道居多（54.54%）,膈下器官多于膈上器官（17∶5）;50.00%（11/22）发生于手术中清除淋巴结。淋巴造血组织肿瘤中的MPMNs的治疗主要为手术治疗和（或）化疗（86.36%,19/22）,其次为对症治疗（13.63%,3/22）。随访12例（55.56%）,6例生存,6例死亡,生存时间3~50个月。结论 淋巴造血组织肿瘤中MPMNs以CLL/SLL和DLBCL多见,近半数淋巴造血组织肿瘤发生于实体肿瘤手术所涉及的区域淋巴结。对MPMNs中构成的淋巴组织肿瘤进行正确组织学分型有助于制定正确、合理的后续治疗方案。对于淋巴造血组织肿瘤中构成的MPMNs的认识尚需要积累更多临床病理研究。

OBJECTIVE Multiple primary malignant neoplasms（MPMNs） was defined as two or more different tissue differentiation of primary malignant tumors which occurred simultaneously or successively in an individual patient. This study was aimed to investigate. To investigate the clinicopathologic features of MPMNs constituted by tumors of hematopoietic and lymphoid tissues. METHODS The clinical and pathologic profiles of 22 cases were evaluated, which were diagnosed in department of pathology, West China hospital, Sichuan University from April 1st 2009 to Jannury 31st 2014. RESULTS Totally 22 cases （2.79%） were scanned out from 7 898 cases of hematopoietic system tumor. Among the 22 cases of MPMNs constituted with byin tumors of hematopoietic and lymphoid tissues,20 cases were synchronous primary tumors,2 cases were metachronous primary tumor. The first tumors were carcinoma （18 cases）, acute myeloid leukemia（AML, 1 case）, non-Hodgkin lymphoma （NHL, 2 cases） and gastrointestinal stromal tumor （GIST, 1 case）.The second tumors were NHL（18 cases） ,classical Hodgkin lymphoma（CHL, 1 case） ,GIST（1 case） and carcinoma （2 cases）. The percentages of B-cell lymphoma and T-cell lymphoma were 86.36%0 （19/22）and 4.54% （1/22） respectively among tumors of hematopoietic and lymphoid tissues constituted with MPMNs. Chronic lymphocytic leukemia/small lymphocytic lymphoma （CLL/SLL, 36. 36%） and diffuse large B-cell lymphoma （DLBCL, 27.27%） were common. More than half of the MPMNs constituted tumors of hematopoietic and lymphoid tissues occurred in gastrointestinal tract,ratio of cases localized in subphrenic organ to that of above diaphragm was 17 ： 5. Half of tumors of hematopoietic and lymph oid tissues were discovered in draining lymph node dissection. Nineteen patients （86.36%） received surgery and/or chemotherapy（or radiotherapy）. Follow-up data was available in 12（55.56%） patients. Six patients died of the tumor. The survival time was 3-50 months. CONCLUSIONS MPMNs constituted by tumors of hematopoietic and lymphoid tissues are rare. CLL/SLL and DLBCL are common. Half of tumors of hematopoietic and lymphoid tissues constituted with MPMNs are found in draining lymph node dissection. Accurate histological classification benefits to sequential treatment. More clinicopathological studies are necessary to define these tumors.