PDF(Pubmed)
Abstract：
Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the appendix or other organ ruptures. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is currently the preferred treatment, with excellent efficacy and safety, and is associated with breakthrough progress in long-term disease control and prolonged survival. However, the high recurrence rate of PMP is the key challenge in its treatment, which limits the clinical application of multiple rounds of CRS-HIPEC and does not benefit from conventional systemic chemotherapy. Therefore, the development of alternative therapies for patients with refractory or relapsing PMP is critical. The literature related to PMP research progress and treatment was searched in the Web of Science, PubMed, and Google Scholar databases, and a literature review was conducted. The overview of the biological research, treatment status, potential therapeutic strategies, current research limitations, and future directions associated with PMP are presented, focuses on CRS-HIPEC therapy and alternative or combination therapy strategies, and emphasizes the clinical transformation prospects of potential therapeutic strategies such as mucolytic agents and targeted therapy. It provides a theoretical reference for the treatment of PMP and the main directions for future research.
摘要：
腹膜假粘液瘤（PMP）是一种罕见的疾病，其特征是在阑尾或其他器官的原发性粘液性肿瘤破裂后广泛的腹膜植入和大量粘液分泌。细胞减灭术(CRS)联合腹腔热灌注化疗(HIPEC)是目前首选的治疗方法,具有优异的疗效和安全性，并与长期疾病控制和延长生存期的突破性进展有关。然而,PMP的高复发率是其治疗的关键挑战，这限制了多轮CRS-HIPEC的临床应用，并且不能从常规的全身化疗中获益。因此,针对难治性或复发性PMP患者开发替代疗法至关重要.有关PMP研究进展和治疗的文献在WebofScience中检索,PubMed,和谷歌学者数据库，并进行了文献综述。生物研究的概述，治疗状态,潜在的治疗策略，当前研究的局限性，并提出了与PMP相关的未来方向，专注于CRS-HIPEC治疗和替代或联合治疗策略，并强调了粘液溶解剂和靶向治疗等潜在治疗策略的临床转化前景。为PMP的治疗提供了理论参考和未来研究的主要方向。
