虽然治疗已取得进展,但恶性胸膜间皮瘤仍然是高致命性恶性肿瘤。总的来说,中位生存时间为 10~15 个月,仅 5%~10% 的患者在诊断后存活 5 年。[34]de Perrot M, Feld R, Cho BC, et al. Trimodality therapy with induction chemotherapy followed by extrapleural pneumonectomy and adjuvant high-dose hemithoracic radiation for malignant pleural mesothelioma. J Clin Oncol. 2009;27:1413-1418.http://www.ncbi.nlm.nih.gov/pubmed/19224855?tool=bestpractice.com[59]Rice DC, Stevens CW, Correa AM, et al. Outcomes after extrapleural pneumonectomy and intensity-modulated radiation therapy for malignant pleural mesothelioma. Ann Thorac Surg. 2007;84:1685-1692.http://www.ncbi.nlm.nih.gov/pubmed/17954086?tool=bestpractice.com
可动手术的疾病
单独使用手术治疗,胸内局部病灶恶化的风险较高。胸膜外肺切除术 (EPP) 加用放疗后的局部失效风险似乎更低,但远处转移更为常见。[59]Rice DC, Stevens CW, Correa AM, et al. Outcomes after extrapleural pneumonectomy and intensity-modulated radiation therapy for malignant pleural mesothelioma. Ann Thorac Surg. 2007;84:1685-1692.http://www.ncbi.nlm.nih.gov/pubmed/17954086?tool=bestpractice.com
有上皮样组织学证据,但无纵隔淋巴结累及且已接受多种治疗措施的患者的长期生存率最好。但是,此亚组的复发和死亡风险仍然很高。[34]de Perrot M, Feld R, Cho BC, et al. Trimodality therapy with induction chemotherapy followed by extrapleural pneumonectomy and adjuvant high-dose hemithoracic radiation for malignant pleural mesothelioma. J Clin Oncol. 2009;27:1413-1418.http://www.ncbi.nlm.nih.gov/pubmed/19224855?tool=bestpractice.com[59]Rice DC, Stevens CW, Correa AM, et al. Outcomes after extrapleural pneumonectomy and intensity-modulated radiation therapy for malignant pleural mesothelioma. Ann Thorac Surg. 2007;84:1685-1692.http://www.ncbi.nlm.nih.gov/pubmed/17954086?tool=bestpractice.com有肉瘤样组织学证据、纵隔淋巴结累及或未能完成多种治疗措施(常见于疾病早期)的患者的预后更糟糕。[34]de Perrot M, Feld R, Cho BC, et al. Trimodality therapy with induction chemotherapy followed by extrapleural pneumonectomy and adjuvant high-dose hemithoracic radiation for malignant pleural mesothelioma. J Clin Oncol. 2009;27:1413-1418.http://www.ncbi.nlm.nih.gov/pubmed/19224855?tool=bestpractice.com[59]Rice DC, Stevens CW, Correa AM, et al. Outcomes after extrapleural pneumonectomy and intensity-modulated radiation therapy for malignant pleural mesothelioma. Ann Thorac Surg. 2007;84:1685-1692.http://www.ncbi.nlm.nih.gov/pubmed/17954086?tool=bestpractice.com
不能动手术或复发性疾病
恶性胸膜间皮瘤无法切除的患者,接受最佳化疗后的中位生存期约 12.3 个月,但疾病进展和死亡不可避免。[48]Vogelzang NJ, Rusthoven JJ, Symanowski J, et al. Phase III study of pemetrexed in combination with cisplatin versus cisplatin alone in patients with malignant pleural mesothelioma. J Clin Oncol. 2003;21:2636-2644.http://www.ncbi.nlm.nih.gov/pubmed/12860938?tool=bestpractice.com局部病灶的进展可导致胸痛、呼吸困难、吞咽困难,最终发育迟滞。远处转移也很常见,可引起部位特异性症状。