CAJ | 학술논문

目的探讨纵隔造血与淋巴组织肿瘤(MTHL)的临床及组织病理学特征、诊断及鉴别诊断.方法按WHO( 2008)造血与淋巴组织肿瘤分类,应用光镜观察、免疫组织化学EnVision法染色、原位杂交对2003年11月至2011年8月收集的40例MTHL进行临床病理学分析.结果 40例MTHL中,男20例,女20例.男女之比为1:1,年龄范围12～70岁.28例伴有胸闷、咳嗽、呼吸困难等上腔静脉综合征等症状.4例为纵隔肿物切除标本,36例为活检标本(其中25例为胸腔镜活检),肿物位于前纵隔20例,后纵隔2例,上纵隔1例,前上纵隔8例,后上纵隔2例,前中纵隔2例,中上纵隔1例.28例有术中冷冻切片检查结果,17例术中诊断为淋巴造血组织肿瘤(冷冻切片和石蜡切片符合率为60.7％).经典型霍奇金淋巴瘤(cHL) 12例(结节硬化型8例,混合细胞型3例,富于淋巴细胞型1例);原发性纵隔大B细胞淋巴瘤(PMBCL) 10例;前体淋巴细胞肿瘤10例[T淋巴母细胞白血病/淋巴瘤(T-LBL)8例;B-LBL 2例];结外黏膜相关淋巴组织边缘区淋巴瘤(MALT淋巴瘤)、复合性淋巴瘤(PMBCL与cHL组合)各1例;髓细胞肉瘤2例;灰区淋巴瘤4例(其中3例形态学类似于cHL,1例类似于DLBCL,均表达CD20、PAX5、CD30,2例表达CD15).11例进行了EBER原位杂交,2例阳性,阳性病例均为cHI.结论 MTHL好发于青少年和年轻患者,前纵隔最为多见.组织学类型以cH-L、PMBCL、T-LBL最常见.灰区淋巴瘤好发于年轻患者,形态学类似于cHL的病例,免疫组织化学类似于PMBCL,而形态学类似于PMBCL的病例,免疫组织化学类似于cHL.胸腔镜活检、术中冷冻切片检查与合理的免疫组织化学抗体组合,是较为可行的诊断途径.
목적 탐토종격조혈여림파조직종류(MTHL)적림상급조직병이학특정、진단급감별진단.방법 안WHO( 2008)조혈여림파조직종류분류,응용광경관찰、면역조직화학EnVision법염색、원위잡교대2003년11월지2011년8월수집적40례MTHL진행림상병이학분석.결과 40례MTHL중,남20례,녀20례.남녀지비위1:1,년령범위12～70세.28례반유흉민、해수、호흡곤난등상강정맥종합정등증상.4례위종격종물절제표본,36례위활검표본(기중25례위흉강경활검),종물위우전종격20례,후종격2례,상종격1례,전상종격8례,후상종격2례,전중종격2례,중상종격1례.28례유술중냉동절편검사결과,17례술중진단위림파조혈조직종류(냉동절편화석사절편부합솔위60.7％).경전형곽기금림파류(cHL) 12례(결절경화형8례,혼합세포형3례,부우림파세포형1례);원발성종격대B세포림파류(PMBCL) 10례;전체림파세포종류10례[T림파모세포백혈병/림파류(T-LBL)8례;B-LBL 2례];결외점막상관림파조직변연구림파류(MALT림파류)、복합성림파류(PMBCL여cHL조합)각1례;수세포육류2례;회구림파류4례(기중3례형태학유사우cHL,1례유사우DLBCL,균표체CD20、PAX5、CD30,2례표체CD15).11례진행료EBER원위잡교,2례양성,양성병례균위cHI.결론 MTHL호발우청소년화년경환자,전종격최위다견.조직학류형이cH-L、PMBCL、T-LBL최상견.회구림파류호발우년경환자,형태학유사우cHL적병례,면역조직화학유사우PMBCL,이형태학유사우PMBCL적병례,면역조직화학유사우cHL.흉강경활검、술중냉동절편검사여합리적면역조직화학항체조합,시교위가행적진단도경.
Objective To study clinical and histopathological features,and diagnosis of mediastinal tumours of haematopoietic and lymphoid tissues(MTHL).Methods Forty cases of MTHL were analyzed for clinicopathology by microscopy and immunohistochemical staining and in situ hybridization,according to the updated 2008 WHO classification of tumours of haematopoietic and lymphoid tissues.Results In 40 cases of MTHL,there were 20 males and 20 females.The ratio of male/female was 1:1.The mean age was 31.8 yeasts and median age was 29 years( range,12-70 years).Superior vena cava syndrome was observed in 28 cases.The specimens of 4 cases were obtained by hmpectomy,whereas 36 cases by biopsy ( 25 cases by thoracoscopy,1 by core needle aspiration).Twenty cases lay in anterior mediastinum,and 2 in posterior,1 in superior,8 in anterior and superior,2 in posterior and superior,2 in anterior and middle,1 in middle and anterior mediastinum.Frozen section were performed in 28 cases,and 17 cases were diagnosed as tumours of haematopoietic and lymphoid tissues ( consistency ratio was 60.7％ ).Twelve cases were classical Hodgkin lymphomas(cHL) (8 were nodular sclerosis subtype,and 3 were mixed cellarity,1 was lymphocyte-rich subtype),and 10 were primary mediastinal ( thymic ) large B cell lymphoma ( PMBCL),10 were precursor lymphocyte neoplasm [ 8 were T lymphoblastic leukemia/lymphomas(T-LBL),2 were B-LBL],1 was MALT lymphoma,1 was composite lynmphoma (PMBCL and cHL),2 were myeloid sarcomas,4 were gray zone lymphomas(GZL) (3 had morphology reminiscent of cHL,and 1 of DLBCL,all cases were positive for CD20,PAX5,CD30 and CD15).EBER were detected in 11 cases by in situ hybridization,2 of which were positive( 18.2％ ),and the 2 positive cases were cHL.Conclusions MTHLs occur predominandy in adolescents and young adults,mainly present as superior vena cava syndrome and anterior mediasinal masses,cHL,PMBCL,T-LBL were the most common MTHLs.GZLs mainly occur in young adults,those whose morphology reminiscent of cHL,immunohistochemistry reminiscent of PMBCL,and vice versa.Thoracoscopy,frozen section and a suitable panel of antibodies were practical approaches to MTHL.