Division of Surgical Oncology, Department of Surgery and the Marlene and Stewart Greenebaum Cancer Center, University of Maryland School of Medicine, Baltimore, MD, USA.
J Natl Compr Canc Netw. 2012 Jan;10(1):49-57. doi: 10.6004/jnccn.2012.0008.
Diffuse malignant peritoneal mesothelioma (DMPM) is a rare and ultimately fatal cancer that was first recognized and described a century ago. It is a diffuse primary malignant condition arising from the mesothelial lining of the peritoneum, and its natural history is hallmarked by a propensity to progress almost exclusively within the abdominal cavity throughout the entire course of disease. Patients afflicted with DMPM most commonly present with nonspecific abdominal symptoms that lead to diagnosis when the condition is relatively advanced. Historically, median overall survival for patients with DMPM without treatment is very short, averaging 6 months. Systemic chemotherapy using pemetrexed and cisplatin has an overall response rate of approximately 25% and a median overall survival of approximately 1 year. Many institutional reports have shown that in selected patients, operative cytoreduction and hyperthermic intraoperative peritoneal chemotherapy using cisplatin or mitomycin C is associated with long-term survival. Recent studies on the molecular biology of DMPM have yielded new insights relating to the potentially important role of the phosphatidylinositol 3-kinase/mammalian target of rapamycin and epidermal growth factor receptor pathways in this disease, which may translate into new therapeutic options for patients with DMPM.
弥漫性恶性腹膜间皮瘤（DMPM）是一种罕见且最终致命的癌症，它在一个世纪前被首次发现并描述。它是一种源于腹膜间皮的弥漫性原发性恶性疾病，其自然病史的特点是疾病的整个过程中几乎仅在腹腔内进展。患有 DMPM 的患者最常见的表现为非特异性腹部症状，当病情相对较严重时才会导致诊断。从历史上看，未经治疗的 DMPM 患者的中位总生存期非常短，平均为 6 个月。使用培美曲塞和顺铂的全身化疗总体缓解率约为 25%，中位总生存期约为 1 年。许多机构报告显示，在选定的患者中，使用顺铂或丝裂霉素 C 的手术细胞减灭术和术中腹腔热化疗与长期生存相关。最近对 DMPM 分子生物学的研究揭示了磷脂酰肌醇 3-激酶/雷帕霉素的哺乳动物靶蛋白和表皮生长因子受体途径在该疾病中的潜在重要作用，这可能为 DMPM 患者带来新的治疗选择。
