64例胸膜恶性间皮瘤的临床病理分析

Malignant pleural mesothelioma: clinicopathologic analysis in a consecutive series of 64 patients from a single institution in China

目的 探讨恶性胸膜间皮瘤（malignant pleural mesothelioma,MPM）的临床病理特征及诊断与鉴别诊断。方法 对64例MPM的临床病理特征、免疫表型、p16基因FISH检测结果进行分析,并复习相关文献。结果 64例MPM中男性43例,女性21例,发病年龄12~78岁。标本获得方式包括手术切除（14/64,21.9%）,胸腔镜下切取活检（18/64,28.1%）及粗针穿刺活检（32/64,50%）。组织学亚型包括上皮样型41例,双相型20例以及肉瘤样型3例。对其中46例MPM及另外24例间皮增生的患者进行p16基因纯合性缺失FISH检测,46例MPM中成功检测29例,发现15例存在p16基因的纯合性缺失（15/29,51.7%）,而24例间皮增生中仅见1例（该例镜下间皮细胞增生十分显著,后经随访证实为间皮瘤）。对15例p16基因纯合性缺失的MPM患者进行随访,其中7例在诊断后的1年内死亡（随访1~12个月）,3例存活至今（随访5~17个月）,中位生存时间9个月,另5例失访。结论 MPM是一种少见但发病率逐年上升的恶性肿瘤,目前的治疗手段效果欠佳,预后差。其组织学形态多样,诊断困难,应用免疫组化对诊断与鉴别诊断有很大帮助,而p16基因纯合性缺失FISH检测则对良、恶性间皮增生的鉴别有重要意义。

Objective To investigate the clinicopathologic characteristics, pathologic diagnosis and differential diagnosis of malignant pleura1 mesothelioma （MPM）. Methods The clinical and histological findings, immunopheuotype, p16 FISH deletion and prognosis of 64 cases of MPMs were evaluated with review of the relevant literature. Results All of 43 patients were male and 21 were female, with age from 12 to 78 years. Specimens from surgical removal（ 14/64,21.9% ） , biopsy by thoracscope （ 18/64,28.1% ）, coarse needle biopsy（32/64, 50%）. There were 41 epithelioid MPMs, 20 biphasic MPMs and 3 sarcomatoid MPMs. p16 FISH was used to investigate 29 MPMs and 24 surface mesothelial proliferation cases successfully, homozygous deletion of p16 was find in 15/29 MPMs and 1/24 mesothelial proliferation cases. Of the 15 patients with p16 homozygous deletion, 7 died in 1 year （followed 1-12 months） and 3 survived till now （followed 5-17 months）, the median survival was 9 months, the other 5 patients lost to follow-up. Conclusions MPM is a rare disease but its incidence is increasing worldwide, there are no efficient cures for this disease by now. It is difficult to diagnose MPM because of its morphological variety, immunohistochemistry could be helpful and p16 deletion FISH is becoming more important in the differentiation between surface mesothelial proliferation and MPM.