The Rutgers Cancer Institute of New Jersey and the Department of Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Ann Surg Oncol. 2018 Aug;25(8):2159-2164. doi: 10.1245/s10434-018-6337-5. Epub 2018 Feb 8.
Diffuse malignant peritoneal mesothelioma (MPM) is a rare and ultimately fatal cancer that was first described just over a century ago. It is a diffuse malignancy arising from the mesothelial lining of the peritoneum; morbidity and mortality from MPM is due to its propensity to progress locoregionally within the abdominal cavity.
The purpose of this article is to review the current state-of-the-science related to the diagnosis, staging, and treatment of MPM.
The condition afflicts men and women equally and the peak incidence is between 55 and 60 years of age although it can arise in the young and elderly. Patients afflicted with MPM most commonly present with nonspecific abdominal symptoms that usually lead to diagnosis when the condition is relatively advanced. Historically, median overall survival for MPM patients without treatment is < 1 year. The couplet of systemic pemetrexed and cisplatin has an overall response rate of approximately 25% and a median overall survival of approximately 1 year.
The available data, almost all retrospective in nature, have shown that in selected patients, operative cytoreduction (CRS) and regional chemotherapy administered as hyperthermic intraoperative peritoneal chemotherapy (HIPEC) or early postoperative intraperitoneal chemotherapy (EPIC) is associated with long-term survival. Studies on the molecular biology of MPM have yielded new insights relating to the potentially important role of the phosphoinsitide-3-kinase/mammalian target of rapamycin (PI3 K/mTOR) pathways and immune checkpoint inhibitors that may translate into new therapeutic options for patients with diffuse MPM.
弥漫性恶性腹膜间皮瘤（MPM）是一种罕见的致命癌症，于一个多世纪前首次描述。它是一种起源于腹膜间皮衬里的弥漫性恶性肿瘤；MPM 的发病率和死亡率归因于其在腹腔内局部进展的倾向。
本文的目的是回顾目前关于 MPM 的诊断、分期和治疗的科学现状。
这种疾病在男性和女性中的发病率相等，发病高峰年龄在 55 至 60 岁之间，尽管它也可能发生在年轻人和老年人中。患有 MPM 的患者最常出现非特异性腹部症状，当病情相对较严重时通常会导致诊断。历史上，未经治疗的 MPM 患者的中位总生存期<1 年。培美曲塞和顺铂的联合治疗总缓解率约为 25%，中位总生存期约为 1 年。
几乎所有的现有数据都是回顾性的，这些数据表明，在选定的患者中，手术细胞减灭术（CRS）和区域化疗作为术中高温腹腔化疗（HIPEC）或术后早期腹腔内化疗（EPIC）与长期生存相关。对 MPM 分子生物学的研究提供了新的见解，涉及磷酸肌醇-3-激酶/雷帕霉素靶蛋白（PI3K/mTOR）途径和免疫检查点抑制剂的潜在重要作用，这可能为弥漫性 MPM 患者带来新的治疗选择。
