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The Journal of the Royal College of... Sep 2023
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms
PubMed: 37329264
DOI: 10.1177/14782715231179254 -
Medicina Clinica Dec 2023
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Adenocarcinoma, Mucinous
PubMed: 37423876
DOI: 10.1016/j.medcli.2023.05.015 -
Frontiers in Oncology 2024Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This...
OBJECTIVE
Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This study aimed to explore the current situation and frontier trend of PMP through bibliometric and visualization analysis, and to indicate new directions for future research.
METHODS
The original research articles and reviews related to the PMP research were downloaded from Web of Science Core Collection on September 11, 2023. CiteSpace (6.2.R4) and VOSviewer(1.6.18) were used to perform bibliometric analysis of the publications, and establish the knowledge map. The data collected was analyzed using the Online Analysis Platform of Bibliometric to evaluate the cooperation of countries in this field.
RESULTS
We identified 1449 original articles and reviews on PMP published between 1998 and 2023. The number of publications on PMP increased continuously. The United States, the United Kingdom and China were the top contributors. The most productive organization was the MedStar Washington Hospital Center. Sugarbaker, Paul H. was the most prolific author and the most cited. Keyword analysis showed that "Pseudomyxoma peritonei", "cancer", "cytoreductive surgery", and "hyperthermic intraperitoneal chemotherapy" were the most common keywords. The earliest and latest used keywords were "mucinous tumors" and "impact", respectively. "classification", "cytoreductive surgery", "appendiceal" were the top 3 strongest citation bursts. The reference "Carr NJ, 2016, " had the highest co-citations.
CONCLUSION
This bibliometric analysis showed an increasing trend in literature related to PMP. The research trends and hotspots identified in this study could guide the future research directions in this field, in order to promote the development of PMP.
PubMed: 38390264
DOI: 10.3389/fonc.2024.1323796 -
Biomedicines Jul 2023In pseudomyxoma peritonei (PMP), and mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: may induce...
In pseudomyxoma peritonei (PMP), and mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: may induce GMCSF expression, while may enhance the expression of cyclic adenosine monophosphate and A2AR signaling. This study aimed to explore possible mechanisms facilitated by and mutations for escaping immune surveillance. Additionally, we looked for new potential therapeutic and prognostic targets in this rare disease which is poorly characterized at the molecular level. GM-CSF, A2AR, CD73, CD39, and PD-L1 expression was investigated by immunohistochemistry in 40 PMPs characterized for and mutational status. Immune cell populations were studied by immunohistochemistry and nanostring nCounter. Following the criteria of a prognostic nomogram reported for PMP, we stratified the patients into two different risk groups, with 28 "low-risk" and 12 "high-risk" patients. We observed the expression of GM-CSF (74%); CD39 (37%); CD73 (53%); A2AR (74%); and PD-L1 (16%) which was unrelated to or status. The tumor microenvironment showed the presence of CD4+ T cells (86%); CD8+ T cells (27%); CD20+ B (67%); CD15+ cells (86%); and CD163+ M2 macrophages (67%), while CD56+ NK cells were absent. CD163 expression (27%) in PMP tumor cells was associated with poor prognosis. mutation and A2AR expression were not associated with a specific immune transcriptional signature. However, the expression assay revealed 21 genes associated with prognosis. The "high-risk" patients exhibited worse progression-free survival (HR = 2.3, CI 95%: 1.1-5.1, = 0.034) and significant downregulation of , , , , , and . In conclusion, we documented the presence of immunosuppressive factors such as GM-CSF, A2AR, and PD-L1 in PMP. These factors were not associated with and status and could be explored as therapeutic molecular targets. Additionally, a set of potential prognostic biomarkers, including CD163 expression in tumor cells, deserve further investigation.
PubMed: 37509688
DOI: 10.3390/biomedicines11072049 -
Cancers Apr 2024Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the appendix or other organ ruptures. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is currently the preferred treatment, with excellent efficacy and safety, and is associated with breakthrough progress in long-term disease control and prolonged survival. However, the high recurrence rate of PMP is the key challenge in its treatment, which limits the clinical application of multiple rounds of CRS-HIPEC and does not benefit from conventional systemic chemotherapy. Therefore, the development of alternative therapies for patients with refractory or relapsing PMP is critical. The literature related to PMP research progress and treatment was searched in the Web of Science, PubMed, and Google Scholar databases, and a literature review was conducted. The overview of the biological research, treatment status, potential therapeutic strategies, current research limitations, and future directions associated with PMP are presented, focuses on CRS-HIPEC therapy and alternative or combination therapy strategies, and emphasizes the clinical transformation prospects of potential therapeutic strategies such as mucolytic agents and targeted therapy. It provides a theoretical reference for the treatment of PMP and the main directions for future research.
PubMed: 38611084
DOI: 10.3390/cancers16071406 -
Chirurgie (Heidelberg, Germany) Oct 2023Disseminated peritoneal seeding of mucin-forming tumors is a rare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the... (Review)
Review
INTRODUCTION
Disseminated peritoneal seeding of mucin-forming tumors is a rare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the prognostic assessment. The past 10 years have led to a standardization of nomenclature and subsequently to the establishment of therapeutic standards. This article aims to provide the current status of the pathological classification, staging, and grading.
METHOD AND MATERIAL
Selective literature search in PubMed and Medline RESULTS: The vast majority of disseminated peritoneal mucinous diseases that correspond to the clinical presentation of pseudomyxoma peritonei (PMP) arise from mucinous tumors of the vermiform appendix. Here are to be distinguished: 1) low-grade appendiceal mucinous neoplasms (LAMN), 2) (very rare) high-grade appendiceal mucinous neoplasms (HAMN), 3) mucinous adenocarcinoma without signet ring cells (G2) and 4) mucinous adenocarcinoma with signet ring cells or signet ring cell carcinoma (G3). Other primary tumors only rarely induce PMP. Terms such as mucocele or mucinous cystadenoma of the appendix correspond to LAMN and should no longer be used. Prognostic distinctions are further made between low-grade PMP, which usually arises from LAMN, and the prognostically less favorable high-grade PMP, which usually arises from mucinous/signet ring cell adenocarcinoma or the rare HAMN. Disseminated peritoneal mucinous disease/PMP must then be further distinguished from prognostically excellent local mucin formation of the peri-appendix region.
DISCUSSION
The currently valid nomenclature, as it has emerged from consensus meetings and in parts has also found its way into the current WHO 2019, has significantly contributed to the fact that the prognosis of patients today can be better estimated and effective forms of treatment could be developed.
PubMed: 37418023
DOI: 10.1007/s00104-023-01926-6 -
Journal of Cancer Research and... Jan 2024Pseudomyxoma peritonei (PMP) is an unusual clinical condition typically presenting with widespread mucinous neoplastic lesions within the peritoneum resulting in gelatin...
BACKGROUND
Pseudomyxoma peritonei (PMP) is an unusual clinical condition typically presenting with widespread mucinous neoplastic lesions within the peritoneum resulting in gelatin material-rich ascites. It was first described by Werth in 1884. Ever since, its clinical presentation, definition, site of origin, and prognosis have been a subject of debate. However, many histopathologic, immunohistochemical, and genetic studies have attempted to locate the primary lesion in the appendix in both genders.
OBJECTIVES
To analyze the histological origin and survival outcomes of pseudomyxoma peritonei in patients treated at a regional cancer center.
MATERIALS AND METHODS
Fifteen cases of PMP were diagnosed during the five-year study period. The demographic and clinicopathological details were retrieved; the slides were reviewed and histological parameters reassessed. Descriptive statistics were used to express proportions. Continuous variables were recorded as mean (SD) or median (IQR). Kaplan-Meier (KM) curve was used to estimate overall survival.
RESULTS
Mean age for PMP was found to be 47.5 years for low grade Mucinous Carcinoma Peritonei (MCP), 54.2 years for high grade MCP, and 58 years for high grade MCP with signet ring cells. Most common overall presentation was abdominal distension in 53.3% (8/15) of cases, followed by acute appendicitis in 20% (3/15) cases. PMP was detected synchronous with the primary tumor in 9/15 cases (60%). Primary lesion in the appendix was grossly identified in 7/15 cases, while it was not explored in the remaining eight cases. Yet, by combined clinical, radiological, histopathological, and immunohistochemical analysis, we identified that most of the cases (14/15) had an appendiceal origin (93.3%). The overall survival for 12 months was 50% and for 18 months was 37%.
CONCLUSION
The surgeon and radiologist may well bear in mind the most common possibility of an appendiceal origin for PMP and resect the appendix, irrespective of the presence of a grossly or radiologically detectable lesions. We emphasize that immunohistochemistry helped to detect the site of origin even when the primary was occult.
PubMed: 38261429
DOI: 10.4103/jcrt.jcrt_191_23 -
Clinical Colorectal Cancer Dec 2023Pseudomyxoma peritonei (PMP) is a rare, slow growing tumor, traditionally considered chemoresistant. The only curative approach is cytoreductive surgery (CRS) followed...
INTRODUCTION
Pseudomyxoma peritonei (PMP) is a rare, slow growing tumor, traditionally considered chemoresistant. The only curative approach is cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC). At disease relapse, or in patients with inoperable disease at diagnosis, no standard treatment has been defined, though nonrandomized series showed promising results with fluoropyrimidine-based regimens.
PATIENTS AND METHODS
We conducted a prospective study in patients with relapsed or unresectable PMP and confirmed disease progression at baseline. Patients received MMC (7 mg/m every 6 weeks, up to a maximum of 4 cycles) plus metronomic capecitabine (625 mg/sqm/day b.i.d.) and bevacizumab (7.5 mg/kg every 3 weeks) until disease progression, unacceptable toxicity, or consent withdrawal. Primary endpoint was progression-free survival (PFS); secondary endpoints were overall survival (OS), overall response rate according to RECIST v1.1 criteria, serum markers response and safety.
RESULTS
Fifteen patients were included. At a median follow-up of 26.1 months (IQR, 17.7-49.6), median PFS was 17.9 months (95% CI, 11.0-NE), with 1-year PFS and OS rates of 73% and 87%. Safety profile was manageable, with only 13% G3/G4 treatment-related adverse events.
CONCLUSION
Metronomic capecitabine, bevacizumab, and MMC are an active regimen in advanced and progressive PMP and favorably compares with historical series.
Topics: Humans; Pseudomyxoma Peritonei; Mitomycin; Bevacizumab; Capecitabine; Peritoneal Neoplasms; Prospective Studies; Hyperthermia, Induced; Disease Progression; Appendiceal Neoplasms
PubMed: 37657955
DOI: 10.1016/j.clcc.2023.08.005 -
Chirurgie (Heidelberg, Germany) Oct 2023Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely.... (Review)
Review
Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely. Perforation of the tumors can result in abdominal mucinous collections, so-called pseudomyxoma peritonei (PMP). The challenge in the treatment of LAMN is the adequate approach to the incidental finding of these tumors. If a mucinous neoplasm is preoperatively suspected in cases of an acute condition, usually appendicitis, it must be weighed up whether a conservative approach is justifiable or whether immediate appendectomy is necessary. If this is the case, an intraoperative perforation of the appendix must be avoided and the complete abdominal cavity must be inspected for mucin deposits. If conservative treatment is possible, further treatment should take place at a specialized center. If the neoplasm is first found incidentally during surgery, perforation of the appendix should also be avoided and the entire abdominal cavity should be inspected for a PMP. If a PMP is present cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) should be performed in a specialized center. If LAMN are found during the postoperative histological work-up, it should be evaluated whether a perforation was present and mucin collections are noted in the surgical report. In the case of LAMN without evidence of a PMP, appendectomy is the adequate treatment. In cases of intra-abdominal mucinous collections, samples should be taken and further treatment should be performed at a center with sufficient expertise. An ileocecal resection or oncological hemicolectomy is not indicated. After adequate treatment, all patients should receive a follow-up using cross-sectional imaging (preferably magnetic resonance imaging, MRI) and determination of the tumor markers CEA, CA 19-9 and CA 125.
Topics: Humans; Peritoneal Neoplasms; Adenocarcinoma, Mucinous; Incidental Findings; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Neoplasms, Cystic, Mucinous, and Serous
PubMed: 37378666
DOI: 10.1007/s00104-023-01910-0