纵隔造血与淋巴组织肿瘤的临床病理分析

Clinicopathologic study of 40 cases of mediastinal tumours of haematopoietic and lymphoid tissues

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—LBL2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cHL。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cHL、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。

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 turnouts 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 yeasrs 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 lumpectomy, 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 lymphoma （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,PAXS,CD30 and CD15）. EBER were detected in 11 cases by in situ hybridization,2 of which were positive（ 18.2% ） , and the 2 positive adolescents and young adults, mainly cases were cHL. Conclusions present as superior vena cava MTHLs occur predominantly in 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, imnmnohistochemistJ7 reminiscent of PMBCL, and vice versa. Thoracoscopy,frozen section and a suitable panel of antibodies were practical approaches to MTHL.