Department of Oncology, The Second Affiliated Hospital of Nanchang University, China.
Jiangxi Key Laboratory of Clinical Translational Cancer Research, China.
Medicine (Baltimore). 2022 Feb 11;101(6):e28785. doi: 10.1097/MD.0000000000028785.
Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.We summarized and evaluated a relatively large population of published PPA cases to assess prognostic factors, diagnostic approaches, treatment methods and clinical outcomes. Using the CNKI, Embase, and PubMed databases, literature published in English and Chinese from 1988 through 2020 was searched using the terms "primary pleural angiosarcoma," "pleural angiosarcoma," and "pleuropulmonary angiosarcoma."A total of 43 patients with PPA were identified in retrospective case series and case reports. The median age at diagnosis was 64 years (range 24-87 years), and the median overall survival was 4 months (range 0.1-180 months). Approximately 80% of patients died from PPA within 10 months of diagnosis, and the 2-year survival rate was approximately 4.4%. In univariate analyses, the presence of pleural effusion and hemothorax were significant predictors of decreased survival, with hazard ratios (HRs) of 2.7 (P = .04) and 3.3 (P = .006), respectively. Sixteen patients received no therapy, and their prognosis was worse than patients who did receive therapy (P = .019). Radiation therapy improved survival more than no radiation therapy (P = .007). Patients appeared to derive clinical benefit from chemotherapy (P = .048). However, tumor resection did not seem to provide a survival benefit (P = .051). In multivariate analysis, tumor resection, and radiation were independent, statistically significant, positive predictors of better survival, with HRs of 0.3 (P = .017) and 0.1 (P = .006), respectively. The presence of hemothorax was an independent predictor of worse prognosis (P = .006).Primary angiosarcoma of the pleura is a rare, poorly understood malignancy with a poor prognosis; hence, the clinical spectrum of PPA is not completely defined. By multivariate analysis, this retrospective study showed a survival benefit of tumor resection or radiation therapy, and the presence of hemothorax was a significant prognostic factor for poor outcomes.
原发性胸膜血管肉瘤（PPA）是一种极其罕见的恶性肿瘤，目前尚无治疗共识。无论采用何种治疗方法，PPA 的临床病程通常都很快导致死亡。我们总结和评估了大量已发表的 PPA 病例，以评估预后因素、诊断方法、治疗方法和临床结局。使用中国知网、Embase 和 PubMed 数据库，以“原发性胸膜血管肉瘤”“胸膜血管肉瘤”和“胸膜肺血管肉瘤”等术语检索了 1988 年至 2020 年发表的英文和中文文献。回顾性病例系列和病例报告共确定了 43 例 PPA 患者。诊断时的中位年龄为 64 岁（范围 24-87 岁），中位总生存期为 4 个月（范围 0.1-180 个月）。大约 80%的患者在诊断后 10 个月内死于 PPA，2 年生存率约为 4.4%。单因素分析显示，胸腔积液和血胸的存在是生存时间缩短的显著预测因素，风险比（HR）分别为 2.7（P=0.04）和 3.3（P=0.006）。16 例患者未接受治疗，其预后比接受治疗的患者差（P=0.019）。放射治疗比不放射治疗更能改善生存（P=0.007）。患者似乎从化疗中获得了临床获益（P=0.048）。然而，肿瘤切除术似乎并不能提供生存获益（P=0.051）。多因素分析显示，肿瘤切除术和放疗是独立的、有统计学意义的生存的正预测因素，风险比分别为 0.3（P=0.017）和 0.1（P=0.006）。血胸的存在是预后不良的独立预测因素（P=0.006）。胸膜原发性血管肉瘤是一种罕见的、尚未完全了解的恶性肿瘤，预后不良；因此，PPA 的临床谱尚不完全明确。通过多因素分析，本回顾性研究显示肿瘤切除术或放疗有生存获益，血胸的存在是预后不良的显著预后因素。
