Abstract
Simple SummaryPseudomyxoma Peritonei (PMP) represents a rare entity which greatly benefits from Cytoreductive Surgery (CRS) associated with Hyperthermic Intraperitoneal Chemotherapy (HIPEC). In fact, CRS-HIPEC represents the treatment with potential chances of cure and long-term disease control of patients affected by PMP. This therapeutic strategy should be performed in referral centers, where a consolidated know-how of this locoregional treatment and a multidisciplinary approach are available. CRS-HIPEC provides excellent results for PMP patients in terms of postoperative outcome, overall and disease-free survival, and quality of life. However, in patients with an extensive or recurrent disease, few therapeutic opportunities are available. This review is focused on the most recent clinical evidence and provides a better understanding of the molecular prognostic factors and potential therapeutic targets in this rare malignancy.Pseudomyxoma Peritonei (PMP) is an anatomo-clinical condition characterized by the implantation of neoplastic cells on peritoneal surfaces with the production of a large amount of mucin. The rarity of the disease precludes the evaluation of treatment strategies within randomized controlled trials. Cytoreductive Surgery (CRS) combined with Hyperthermic Intraperitoneal Chemotherapy (HIPEC) has proven to be the only therapeutic option with potential chances of cure and long-term disease control. The present review discusses the epidemiology, pathogenesis, clinical presentation and treatment of PMP, focusing on the molecular factors involved in tumor progression and mucin production that could be used, in the upcoming future, to improve patient selection for surgery and to expand the therapeutic armamentarium.
Highlights
The aim of this study is to review the more controversial aspects of Pseudomyxoma Peritonei (PMP) treatment with a focused perspective on the ongoing clinical and molecular research in this field
low-grade appendiceal mucinous neoplasm (LAMN) treated outside a referral institution but evaluated by a peritoneal cancer center according to an internal protocol of pathology review and follow-up, the occurrence of PMP was only 2% [21]
Overexpression of p53 in tumor samples was linked to poor survival in a cohort of 194 PMP patients, whereas KRAS mutation did not show a prognostic value in a smaller group of these patients [52]. dMMR and mucin staining were analyzed for prognostic significance, in addition to Ki67 labeling index, altered p53 staining and clinical variables including grading, lymph node involvement, angiolymphatic and perineural invasion
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Because of its indolent course, historical treatment of PMP consists of serial non-radical debulking surgeries, essentially aimed at controlling symptoms and complications [2]. Though CRS-HIPEC is considered the gold standard of treatment for PMP, several questions still remain unanswered and highly debated In this perspective, clinical research in PMP is difficult because of the rarity of the disease, and randomized controlled trials in humans are practically impossible to arrange [9]. Every effort should be done for ameliorating the outcome of patients selected for CRS-HIPEC, mainly through the standardization of the procedure and quality control. Basic research of the biomolecular factors involved in PMP tumor growth and mucin production could, in the future, improve outcomes after optimal radical surgery, as well as reduce symptoms and complications in patients with unresectable disease.
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