增生性恶性胸膜间皮瘤(DMM)是弥漫性恶性胸膜间皮瘤(MPM)的少见组织学亚型,占病例的5-10%。预后不良,直接侵入胸壁或肺和远处转移。它的病理特征包括致密的胶原纤维,呈层状。其预处置病理诊断困难,纤维状胸膜炎和反应性间皮增生作为潜在的鉴别诊断。
我们回顾性回顾了1996年至2012年MPM患者的病历。在60例MPM患者中,确定了四名具有促纤维化亚型的患者及其临床特征,包括接触石棉,治疗,和预后,被审查了。所有患有DMM的患者都是男性,年龄中位数为69岁(范围:63-74岁)。所有四名患者都接触过石棉。在所有4例患者中,对组织学进行了明确诊断,并进行了国际间皮瘤兴趣小组分类(III/IV:2/3)。3例患者接受了化疗(2例接受顺铂/培美曲塞治疗,1例接受顺铂/吉西他滨治疗),1例患者接受了手术。DMM患者的中位生存时间为3.8个月(范围:0.9-11.5个月)。与我们机构的其他亚型MPM患者的10.5个月相比。
DMM继续具有不良预后。识别这种变异并将其与胸膜斑块区分开来是很重要的,非特异性反应性胸膜纤维化,胸膜炎,和其他肺部疾病。
Desmoplastic malignant pleural mesothelioma (DMM) is rare histological subtype of diffuse malignant pleural mesothelioma (MPM), accounting for 5-10 % of cases. It has a poor prognosis, with direct invasion of the chest wall or lungs and distant metastases. Its pathological characteristics include dense collagen fibers in a storiform pattern. Its pretreatment pathological diagnosis is difficult, with fibrous pleuritis and reactive mesothelial hyperplasia as potential differential diagnoses.
We retrospectively reviewed the medical charts of patients with MPM from 1996 to 2012. Among 60 patients with MPM, four patients with the
desmoplastic subtype were identified and their clinical characteristics, including asbestos exposure, treatment, and prognosis, were reviewed. All of the patients with DMM were men, with a median age of 69 years (range: 63-74 years). All four patients had been exposed to asbestos. The definitive diagnosis was made histologically and the International Mesothelioma Interest Group classification was advanced (III/IV: 2/3) in all four patients. Three patients were treated with chemotherapy (two with cisplatin/pemetrexed and one with cisplatin/gemcitabine) and one patient underwent surgery. The median survival time in the patients with DMM was 3.8 months (range: 0.9-11.5 months), compared with 10.5 months in patients with other subtypes of MPM in our institution.
DMM continues to have a poor prognosis. It is important to recognize this variant and distinguish it from pleural plaques, non-specific reactive pleural fibrosis, pleurisy, and other lung diseases.