Pseudomyxoma peritonei: current chemotherapy and the need for mucin-directed strategies

Abstract

Introduction: Pseudomyxoma peritonei (PMP) is a rare neoplastic disease characterized by dissemination of mucinous tumor implants and accumulation of mucin in the abdominopelvic cavity. PMP is a progressive, recurrent condition that dramatically impacts quality of life. The current standard of care includes cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC).

Areas covered: The clinicopathological features of PMP closely correspond with multifocal distribution of mucin-secreting tumors and mucin collections. Hence, maximal elimination of all gross disease is attempted in the treatment of PMP. However, PMP is progressive and recurrent. In this review, current chemotherapy for PMP and a number of experimental strategies for enhancement of microscopic cytoreduction are addressed.

Expert opinion: According to the current standard of care, HIPEC is the chemotherapy of choice for patients with PMP, which is delivered after completion of CRS. This multimodal approach offers long-term benefits for selected patients. Nevertheless, HIPEC fails to maintain the surgical complete response achieved by CRS and needs to be supplemented by novel strategies. Given the role of mucin and mucin-secreting cells in the pathophysiology of PMP, locoregional use of safe agents capable of inducing cytotoxic effects on residual tumor cells and eliminating mucin is a promising avenue for further investigation.

Keywords:

• chemotherapy
• hyperthermic intraperitoneal chemotherapy
• MUC2
• mucin
• pseudomyxoma peritonei

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

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