Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Nov 27;13(23):5965.
doi: 10.3390/cancers13235965.

Novel Perspectives in Pseudomyxoma Peritonei Treatment

Antonio Sommariva  1 , Marco Tonello  1 , Giulia Rigotto  2 , Nayana Lazzari  2 , Pierluigi Pilati  1 , Maria Luisa Calabrò  2
Affiliations
Review

Novel Perspectives in Pseudomyxoma Peritonei Treatment

Antonio Sommariva et al. Cancers (Basel). .

Abstract

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.

Keywords: HIPEC; Pseudomyxoma Peritonei; cytoreductive surgery; mucin; peritoneal metastases.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Intraoperative view of Pseudomyxoma Peritonei.
Figure 2
Figure 2
Main pathways involved in mucin expression in PMP. In the context of chronic inflammation, several factors, including pleiotropic cytokines, growth factors, hormones and LPS, may induce a phosphorylation cascade involving the Ras/Raf/MEK/ERK/RSK and JAK/STAT pathways, and their crosstalk, leading to SP1-, NF-Kb- and STAT-mediated activation of mucin promoters. The 11p15 mucin gene cluster contains four mucin genes, among which MUC2, MUC5AC and MUC5B, i.e., the genes mainly involved in mucin formation in PMP. Mucin mRNAs generate the core protein structures (apomucin) that are glycosylated, assembled and secreted through secretory vesicles. The three main mucins secreted by PMP are gel-forming mucins.
See this image and copyright information in PMC

References

    1. Werth K. Klinische und anatomische Untersuchungen zur Lehre von den Bauchgeschwülsten und der Laparatomie. Arch. Gynäkol. 1884;24:100–118. doi: 10.1007/BF01837425. - DOI
    1. Miner T.J., Shia J., Jaques D.P., Klimstra D.S., Brennan M.F., Coit D.G. Long-term survival following treatment of pseudomyxoma peritonei: An analysis of surgical therapy. Ann. Surg. 2005;241:300–308. doi: 10.1097/01.sla.0000152015.76731.1f. - DOI - PMC - PubMed
    1. Sugarbaker P.H. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7:69–76. doi: 10.1016/S1470-2045(05)70539-8. - DOI - PubMed
    1. Kusamura S., Barretta F., Yonemura Y., Sugarbaker P.H., Moran B.J., Levine E.A., Goere D., Baratti D., Nizri E., Morris D.L., et al. The Role of Hyperthermic Intraperitoneal Chemotherapy in Pseudomyxoma Peritonei After Cytoreductive Surgery. JAMA Surg. 2021;156:e206363. doi: 10.1001/jamasurg.2020.6363. - DOI - PMC - PubMed
    1. Chua T.C., Al-Mohaimeed K., Liauw W., Morris D.L. Pseudomyxoma peritonei: A need to establish evidence-based standard of care--is this the right trial? Ann. Surg. Oncol. 2009;16:2675–2677. doi: 10.1245/s10434-009-0550-1. - DOI - PMC - PubMed

LinkOut - more resources