Pseudomyxoma Peritonei 2021: State of the Art and Trends for the Future in Tumor Biology, Treatment and Outcomes

A special issue of Current Oncology (ISSN 1718-7729).

Deadline for manuscript submissions: closed (31 March 2023) | Viewed by 5089

Special Issue Editors


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Guest Editor
Hospital Moisès Broggi, 08970 Barcelona, Spain
Interests: pseudomyxoma peritonei; cytoreductive surgery; peritoneum tumor, gastroenteropancreatic neuroendocrine tumor; neuroendocrine carcinoma; carcinoid tumor

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Guest Editor
Wake Forest School of Medicine, Wake Forest University, Winston Salem, NC 27101, USA
Interests: tumor organoid technology; pseudomyxoma peritonei; cytoreductive surgery; peritoneum tumor

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Guest Editor
Department of GI Surgery and Cancer Research Institute Ghent (CRIG), Ghent University Hospital, 9000 Ghent, Belgium
Interests: colorectal cancer; peritoneal metastasis; intraperitoneal drug delivery; functional imaging in cancer; biophysics of tumor tissue; drug delivery modelling
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Special Issue Information

Dear Colleagues,

Pseudomyxoma peritonei (PMP) is a rare and enigmatic clinical syndrome characterized by the accumulation of mucinous tumor in the abdominal cavity. It is defined by a biologically, rather indolent, but still uniformly progressive growth of peritoneal metastases that typically arise from a non-invasive primary mucinous appendiceal neoplasm. The distinct biological and clinical characteristics of this syndrome have allowed for the development of a new treatment paradigm for peritoneal metastases based on surgical resection and local–regional chemotherapy. The therapeutic paradigm of cytoreductive surgery and heated intraperitoneal chemotherapy (CRS and HIPEC) has revolutionized not only our understanding and treatment of PMP but of most peritoneal metastases with a major clinical impact in many common cancers. Yet, despite the enormous success of this approach for PMP in terms of survival outcomes, pseudomyxoma peritonei remains a uniquely challenging entity in terms of fully understanding its biology and the prognosis of each individual patient. The creation of treatment units specialized in CRS and HIPEC has allowed for a significant increase in research focused on PMP and has resulted in a much-improved understanding of its biology, clinical presentation, surgical treatment and outcomes. It is also allowing new frontiers of investigation to open up, including molecular characterization, preclinical animal model and organoid development and non-surgical treatment of advanced unresectable disease.

The aim of this Special Issue is to summarize the current accumulated knowledge on the biology, treatment and outcomes of pseudomyxoma peritonei since the introduction of CRS and HIPEC as the standard therapeutic approach and to present future trends in the research and clinical management of this entity.

Dr. Lana Bijelic
Prof. Dr. Konstantinos Ioannis Votanopoulos
Prof. Dr. Wim P. Ceelen
Guest Editors

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Keywords

  • pseudomyxoma peritonei
  • mucinous carcinoma peritonei
  • peritoneal metastases
  • cytoreductive surgery
  • heated intraperitoneal chemotherapy
  • appendix
  • LAMN
  • mucinous adenocarcinoma
  • appendiceal cancer
  • mucinous neoplasm

Published Papers (2 papers)

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Article
Retrospective Review of Outcomes in Non-Invasive Mucinous Appendiceal Neoplasms with and without Peritoneal Spread: A Cohort Study
by Arkadii Sipok, Jonathan M. Dort, Anthony Visioni and Lana Bijelic
Curr. Oncol. 2022, 29(12), 9125-9134; https://doi.org/10.3390/curroncol29120714 - 23 Nov 2022
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Abstract
Patients treated surgically for local non-invasive mucinous appendiceal neoplasm (NI-MAN) may recur with the development of peritoneal dissemination (PD). The risk of recurrence and predictive factors are not well studied. Patients with NI-MAN, with or without peritoneal dissemination at presentation, were included. Patients [...] Read more.
Patients treated surgically for local non-invasive mucinous appendiceal neoplasm (NI-MAN) may recur with the development of peritoneal dissemination (PD). The risk of recurrence and predictive factors are not well studied. Patients with NI-MAN, with or without peritoneal dissemination at presentation, were included. Patients with limited disease underwent surgical resection only. Patients with peritoneal dissemination underwent cytoreductive surgery (CRS) with or without hyperthermic intraperitoneal chemotherapy (HIPEC). Patients without PD (nPD) were compared to those who presented with PD. Thirty-nine patients were included, 25 in nPD and 14 in PD. LAMN was diagnosed in 96% and 93% of patients in nPD and PD, respectively. Acellular mucin on the peritoneal surface was seen in 16% of nPD patients vs. 50% of PD patients (p = 0.019). Two (8%) patients in the nPD group who had LAMN without wall rupture recurred, at 57 and 68 months, with a PCI of 9 and 22. The recurrence rate in the PD group was 36%. All recurred patients underwent CRS+HIPEC. A peritoneal recurrence is possible in NI-MANs confined to the appendix even with an intact wall at initial diagnosis. The peritoneal disease may occur with significant delay, which is longer than a conventional follow-up. Full article

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Review
The Role of Microorganisms in Appendiceal Pseudomyxoma Peritonei: A Review
by Yekaterina Khamzina, Mary Caitlin King, Carol Nieroda, D. Scott Merrell, Armando Sardi and Vadim Gushchin
Curr. Oncol. 2022, 29(5), 3576-3584; https://doi.org/10.3390/curroncol29050289 - 16 May 2022
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Abstract
Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the accumulation of mucinous ascites. PMP represents a spectrum of low to high-grade disease. Despite aggressive management, many PMP [...] Read more.
Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the accumulation of mucinous ascites. PMP represents a spectrum of low to high-grade disease. Despite aggressive management, many PMP patients recur, leading to debilitating symptoms and few treatment options. Therefore, scientists have continued to look for ways to improve treatment and further understand disease pathogenesis. Microorganisms were previously hypothesized to play a role in PMP progression and development. Hence, antibacterial treatment was suggested by some authors, but the data were limited. In this paper, we review the current data on the role of bacteria in PMP, discuss the significance, and suggest possible solutions to the inherent challenges in these studies. Given the limitations of the discussed studies, we remain skeptical about introducing novel antibacterial treatment into clinical practice at this time; however, the available data are valuable and indicate that more research into the molecular mechanisms of PMP is needed. Full article
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