Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (914 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A peritoneal adenocarcinoma characterized by build-up of mucus in the peritoneal cavity. Mucus secreting cells may attach to the peritoneal lining and continue to secrete mucus. The majority of cases represent tumor spread from a primary low-grade mucinous neoplasm of the appendix (NCI Thesaurus).
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Ascites, Gelatinous; Gelatinous Ascites; Syndrome of Pseudomyxoma Peritonei; Pseudomyxoma Peritonei Syndrome; Pseudomyxoma Peritonei Syndromes.
Where it fits in the MeSH classification
- Broader topics
- Adenocarcinoma, Mucinous
Related concepts
- Mucus
- Peritoneal Cavity
- Appendix
Research landscape
PubMed indexes 1,313 records tagged with the MeSH heading “Pseudomyxoma Peritonei” (counts retrieved 2026-10-29). 308 of them (23.5%) were published from 2020 onward, and the busiest period so far is 2010-2019 with 410 records.
Reviews account for 203 records (15.5%) and meta-analyses for 3. Randomized controlled trials number 7 (0.5%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 269 |
| 2000-2009 | 326 |
| 2010-2019 | 410 |
| 2020 onward | 308 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 203 | 15.5% |
| Meta-analyses | 3 | 0.2% |
| Randomized controlled trials | 7 | 0.5% |
Method: PubMed query "Pseudomyxoma Peritonei"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in cancer and oncology
Cancer research spans very different kinds of evidence. Laboratory studies in cell lines and animal models reveal mechanisms but often do not translate to patients. Early-phase trials mainly test safety and dosing, while phase 3 randomized trials and their meta-analyses are the usual basis for treatment conclusions. Outcomes also differ in meaning: tumour shrinkage, progression-free survival and overall survival are not interchangeable, and screening studies can be distorted by lead-time and overdiagnosis. When reading a result, check the cancer type and stage studied, the comparison treatment, and which outcome was measured.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Molecular profiles of high-grade and low-grade pseudomyxoma peritonei | 2015 | Research article Design not recorded in structured metadata | 55 | cc by PMID 26475379 · DOI · PMC5123786 |
| Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines | 2021 | Review article Design not recorded in structured metadata | 54 | cc by PMID 33581733 · DOI · PMC7881689 |
| Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method | 2021 | Research article Design not recorded in structured metadata | 50 | cc by PMID 32488520 · DOI · PMC7752784 |
| Peritoneal metastasis histological grade independently predicts outcome in pseudomyxoma peritonei treated with curative surgery | 2026 | Research article Design not recorded in structured metadata | 2 | cc by PMID 41559428 · DOI · PMC13282280 |
Molecular profiles of high-grade and low-grade pseudomyxoma peritonei [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Pseudomyxoma peritonei (PMP) is a rare disease exhibiting a distinct clinical feature caused by cancerous cells that produce mucinous fluid in the abdominal cavity.
- Closing excerpt
- These findings may be useful for the understanding of tumor characteristics, and facilitate the development of therapeutic strategies.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (235 words)
Pseudomyxoma peritonei (PMP) is a rare disease exhibiting a distinct clinical feature caused by cancerous cells that produce mucinous fluid in the abdominal cavity. PMPs originate most frequently from the appendix and less frequently from the ovary. This disease can range from benign to malignant, and histologically, PMP is classified into two types: disseminated peritoneal adenomucinosis (DPAM) representing the milder phenotype, and peritoneal mucinous adenocarcinomas (PMCA) representing the aggressive phenotype. Although histological classification is clinically useful, the pathogenesis of PMP remains largely unknown. To elucidate the molecular mechanisms underlying PMP, we analyzed 18 PMP tumors comprising 10 DPAMs and 8 PMCAs. DNA was extracted from tumor and matched non-tumorous tissues, and was sequenced using Ion AmpliSeq Cancer Panel containing 50 cancer-related genes. Analysis of the data identified a total of 35 somatic mutations in 10 genes, and all mutations were judged as pathological mutations. Mutations were frequently identified in KRAS (14/18) and GNAS (8/18). Interestingly, TP53 mutations were found in three of the eight PMCAs, but not in the DPAMs. PIK3CA and AKT1 mutations were also identified in two PMCAs, but not in the DPAMs. These results suggested that KRAS and/or GNAS mutations are common genetic features of PMP, and that mutations in TP53 and/or genes related to the PI3K-AKT pathway may render malignant properties to PMP. These findings may be useful for the understanding of tumor characteristics, and facilitate the development of therapeutic strategies.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 26475379 · DOI · PMC5123786
Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines [2]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundPseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people.
- Closing excerpt
- However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (259 words)
BackgroundPseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.Main bodyAs a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS) + hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS + HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS + HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS + HIPEC on PMP.ConclusionCurrently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 33581733 · DOI · PMC7881689
Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method [3]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundThe determination of the incidence and prevalence of rare diseases is important for economists and health-care providers.
- Closing excerpt
- Based on our calculations, a significant number of people are living with PMP in Europe, underlining the need for appropriate resource allocation to ensure that adequate health-care measures are provided.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (233 words)
BackgroundThe determination of the incidence and prevalence of rare diseases is important for economists and health-care providers. Pseudomyxoma peritonei (PMP) is a rare, slow-growing abdominal cancer that represents a substantial burden on both patients and health-care systems. The incidence rate was previously approximated at 1-2 people per million per year; this incidence has never been challenged, and the prevalence has not been estimated.MethodsEpidemiological data from Norway and England were obtained and analysed to calculate a minimum incidence rate based on the number of patients having a first surgical intervention for PMP. A novel method was then used to determine a prevalence rate for PMP, incorporating incidence, death, and cure rates in a multi-year analysis that accounted for the increasing population of Europe over a 10-year period.ResultsAn incidence rate of 3.2 people per million per year was calculated, with a corresponding estimated prevalence rate of 22 people per million per year. By this calculation, 11,736 people in Europe were estimated to be living with PMP in 2018.ConclusionIncidence and prevalence are essential tools for assessment of the financial and human cost of a disease. For rare diseases, such as PMP, the lack of accurate registries presents a particular challenge in determining such health-related statistical parameters. Based on our calculations, a significant number of people are living with PMP in Europe, underlining the need for appropriate resource allocation to ensure that adequate health-care measures are provided.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 32488520 · DOI · PMC7752784
Peritoneal metastasis histological grade independently predicts outcome in pseudomyxoma peritonei treated with curative surgery [4]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundPseudomyxoma Peritonei is characterised by peritoneal metastasis from appendiceal mucinous neoplasms (AMN).
- Closing excerpt
- 21, p DiscussionPeritoneal metastasis histopathological grade predicts outcome for patients with PMP from AMN following CRS + HIPEC independent of primary histology.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (187 words)
BackgroundPseudomyxoma Peritonei is characterised by peritoneal metastasis from appendiceal mucinous neoplasms (AMN). The PSOGI classification (2016) categorises PMP into acellular mucin (AM), low-grade mucinous carcinoma peritonei (LGMCP), and high-grade mucinous carcinoma peritonei (HGMCP). This study aimed to determine long-term prognosis based on this classification.Materials and methodsPathology review from PMP patients with AMNs undergoing cytoreductive surgery and heated intraperitoneal chemotherapy (CRS + HIPEC) with curative intent over a 15-year period (2006-2021) was undertaken. Patients underwent standardised surveillance. Cox proportional hazards regression models, log-rank test, and Kaplan-Meier method were used to assess overall (OS) and disease-free survival (DFS) based on histopathological peritoneal metastasis grade. DFS was only calculated for patients who had a complete cytoreduction.Results290 PMP patients were identified (AM = 34%, LGMCP = 59%, HGMCP = 7%) with median follow-up of 49 months. Median age was 59 years (range: 22-79), M: F of 1:2.5, peritoneal cancer index median of 18 (range: 0-39). Univariate OS hazard ratio (HR) is 2.75 for LGMCP vs AM (95% CI: 1.05 -7.21, p DiscussionPeritoneal metastasis histopathological grade predicts outcome for patients with PMP from AMN following CRS + HIPEC independent of primary histology.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41559428 · DOI · PMC13282280
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process [5]
- Research article New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? [6]
- Research article A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei [7]
- Research article Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment [8]
- Research article Pseudomyxoma peritonei syndrome [9]
- Research article The etiology, clinical presentation, and management of pseudomyxoma peritonei [10]
References
- Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, et al.. Molecular profiles of high-grade and low-grade pseudomyxoma peritonei. . 2015. PMID 26475379 · DOI · PMC5123786
- Lin YL, Xu DZ, Li XB, Yan FC, Xu HB, Peng Z, et al.. Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines. . 2021. PMID 33581733 · DOI · PMC7881689
- Patrick-Brown TDJH, Carr NJ, Swanson DM, Larsen S, Mohamed F, Flatmark K. Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method. . 2021. PMID 32488520 · DOI · PMC7752784
- Hasan A, Chakrabarty B, Kearsey C, Nagaraju R, Wilson M, Renehan A, et al.. Peritoneal metastasis histological grade independently predicts outcome in pseudomyxoma peritonei treated with curative surgery. . 2026. PMID 41559428 · DOI · PMC13282280
- Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, et al.. A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol. 2016. PMID 26492181 · DOI
- Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?. Lancet Oncol. 2006. PMID 16389186 · DOI
- Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2007. PMID 17054002 · DOI
- Govaerts K, Lurvink RJ, De Hingh IHJT, Van der Speeten K, Villeneuve L, Kusamura S, et al.. Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. Eur J Surg Oncol. 2021. PMID 32199769 · DOI
- Sugarbaker PH, Ronnett BM, Archer A, Averbach AM, Bland R, Chang D, et al.. Pseudomyxoma peritonei syndrome. Adv Surg. 1996. PMID 8960339
- Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 2003. PMID 14567019 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.