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Postgraduate Medical Journal Mar 2002Pseudomyxoma peritonei is a relatively rare and poorly understood condition in which mucus accumulates within the peritoneal cavity. The presence of cells in the mucin,...
Pseudomyxoma peritonei is a relatively rare and poorly understood condition in which mucus accumulates within the peritoneal cavity. The presence of cells in the mucin, either inflammatory or neoplastic, distinguishes it from simple acellular mucus ascites caused by mucinous spillage. There is widespread seeding of the peritoneal and omental surfaces with a heavy cancerous glaze. This is principally a complication of borderline or malignant neoplasm of the ovary and/or appendix. This paper describes two cases of previously healthy women who both presented with an acute abdomen, and were diagnosed postoperatively with pseudomyxoma peritonei. In addition, literature on the clinical presentation, diagnostic procedures, and treatment options has been briefly reviewed.
Topics: Abdomen, Acute; Female; Humans; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 11884702
DOI: 10.1136/pmj.78.917.170 -
BMC Cancer Jan 2023To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP)...
BACKGROUND
To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP) patients relative to their diagnostic and predictive value.
METHODS
The study comprised 183 patients with pathologically confirmed appendiceal PMP, enrolled from May 2012 to June 2020, in Aerospace Center Hospital. Serum and ascites tumor markers were obtained, and their diagnostic values were compared by receiver operating characteristic (ROC) curves. The prognostic factors of appendiceal PMP with different pathologic subgroups were calculated by univariate and multivariate Cox proportional hazard regression models.
RESULTS
There were significant differences between the numbers of patients with positive CEA and CA199 in serum vs. ascites: p = 0.034 in CEA and p = 0.006 in CA199, respectively. The sensitivities with optimal cut-off values for ascites markers of CEA, CA199 and CA125 were 83.5%, 88.9% and 72.6%, respectively. CEA in ascites showed significant difference in the diagnosis of appendiceal PMP (p = 0.000); the areas under the ROC curves (AUROCs) and specificity were 0.725, 70.7%, respectively. Univariate analysis showed that the higher the ascites tumor markers, the poorer the survival (p = 0.014). Multivariate analysis indicated that completeness of cytoreduction (CCR), ascites CEA and pathological grade were independent risk factors for overall survival (OS).
CONCLUSION
CEA in ascites can be used to help specify the origin of PMP. Furthermore, elevation of ascites CEA, high pathological grade and incomplete cytoreduction predicted poor prognosis of appendiceal PMP.
Topics: Humans; Pseudomyxoma Peritonei; Prognosis; Carcinoembryonic Antigen; Biomarkers, Tumor; Peritoneal Neoplasms; Ascites; Appendiceal Neoplasms; CA-125 Antigen
PubMed: 36703100
DOI: 10.1186/s12885-023-10545-7 -
Archives of Pathology & Laboratory... Oct 2011Appendiceal mucinous neoplasms are considered enigmatic tumors of unpredictable biologic potential. Their importance lies in their potential to spread to the peritoneum... (Review)
Review
CONTEXT
Appendiceal mucinous neoplasms are considered enigmatic tumors of unpredictable biologic potential. Their importance lies in their potential to spread to the peritoneum and viscera in the form of gelatinous mucin deposits. Extra-appendiceal spread of these tumors is the most common etiology of pseudomyxoma peritonei , which is a descriptive term encompassing a number of neoplastic and nonneoplastic peritoneal disorders. Many studies aimed at evaluating the biologic importance of appendiceal mucinous neoplasms and pseudomyxoma peritonei have employed inconsistent histologic criteria for their diagnosis and descriptive terminology for their classification. As a result, appendiceal mucinous neoplasms and associated peritoneal disease represents one of the most confusing and controversial areas in gastrointestinal pathology.
OBJECTIVES
To summarize the literature regarding the biologic potential of appendiceal mucinous neoplasms and pseudomyxoma peritonei and to discuss the similarities and differences between proposed systems for their classification.
DATA SOURCES
Literature review and case-derived material.
CONCLUSIONS
Many studies have contributed to an increased understanding of the natural progression of mucinous neoplasms of the appendix and peritoneum, and the adoption of a uniform reporting system, as advocated by the American Joint Committee on Cancer and the World Health Organization, will facilitate clear communication among pathologists and clinical colleagues.
Topics: Adenocarcinoma, Mucinous; Appendiceal Neoplasms; Female; Humans; Male; Mucins; Neoplasms, Cystic, Mucinous, and Serous; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 21970481
DOI: 10.5858/arpa.2011-0034-RA -
World Journal of Gastrointestinal... Jun 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard treatment. However, there are few studies and insufficient evidence regarding systemic chemotherapy of advanced PMP. Regimens for colorectal cancer are often used clinically, but there is no uniform standard for late-stage treatment.
AIM
To determine if bevacizumab combined with cyclophosphamide and oxaliplatin (Bev+CTX+OXA) is effective for treatment of advanced PMP. The primary study endpoint was progression-free survival (PFS).
METHODS
Retrospective analysis was conducted on the clinical data of patients with advanced PMP who received Bev+CTX+OXA regimen (bevacizumab 7.5 mg/kg ivgtt d1, oxaliplatin 130 mg/m ivgtt d1 and cyclophosphamide 500 mg/m ivgtt d1, q3w) in our center from December 2015 to December 2020. Objective response rate (ORR), disease control rate (DCR) and incidence of adverse events were evaluated. PFS was followed up. Kaplan-Meier method was used to draw survival curve, and log-rank test was used for comparison between groups. Multivariate Cox proportional hazards regression model was used to analyze the independent influencing factors of PFS.
RESULTS
A total of 32 patients were enrolled. After 2 cycles, the ORR and DCR were 3.1% and 93.7%, respectively. The median follow-up time was 7.5 mo. During the follow-up period, 14 patients (43.8%) had disease progression, and the median PFS was 8.9 mo. Stratified analysis showed that the PFS of patients with a preoperative increase in CA125 (8.9 2.1, = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 5.0, = 0.043) was significantly longer than that of the control group. Multivariate analysis showed that a preoperative increase in CA125 was an independent prognostic factor for PFS (HR = 0.245, 95%CI: 0.066-0.904, = 0.035).
CONCLUSION
Our retrospective assessment confirmed that the Bev+CTX+OXA regimen is effective in second- or posterior-line treatment of advanced PMP and that adverse reactions can be tolerated. A preoperative increase in CA125 is an independent prognostic factor of PFS.
PubMed: 37405093
DOI: 10.4240/wjgs.v15.i6.1149 -
Archives of Pathology & Laboratory... Jun 2010Low grade appendiceal mucinous neoplasms can spread to the peritoneum as pseudomyxoma peritonei even though they are not obviously invasive in the appendix. During the... (Review)
Review
Low grade appendiceal mucinous neoplasms can spread to the peritoneum as pseudomyxoma peritonei even though they are not obviously invasive in the appendix. During the past several decades, several problematic issues surrounding this enigmatic tumor have been debated in the literature, including appropriate nomenclature for the appendiceal tumors and their peritoneal metastases. In this article, the most contentious issues in the area of appendiceal mucinous tumors are examined. First, the classification systems that have been proposed for these tumors are compared in the context of whether the appendiceal mucinous tumors are ruptured adenomas or invasive carcinomas. The controversy about the nature of pseudomyxoma peritonei and its classification systems is discussed in the following section. A brief discussion follows that examines the issue of localized pseudomyxoma peritonei and its clinical significance. Next reviewed is the largely resolved controversy about whether ovarian mucinous tumors in this setting are separate primaries or are metastases from the appendiceal tumor. Finally, the controversy about the most effective treatment of patients with pseudomyxoma peritonei is discussed.
Topics: Adenocarcinoma, Mucinous; Appendiceal Neoplasms; Diagnosis, Differential; Female; Humans; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 20524864
DOI: 10.5858/134.6.864 -
Cancer Medicine Dec 2015Pseudomyxoma peritonei (PMP) is a rare disease exhibiting a distinct clinical feature caused by cancerous cells that produce mucinous fluid in the abdominal cavity. PMPs...
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.
Topics: Aged; Aged, 80 and over; Biomarkers; Combined Modality Therapy; DNA Mutational Analysis; Female; Gene Expression Profiling; Humans; Immunohistochemistry; Male; Middle Aged; Mutation; Neoplasm Grading; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Transcriptome; Tumor Suppressor Protein p53
PubMed: 26475379
DOI: 10.1002/cam4.542 -
BJS Open Apr 2019Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard... (Observational Study)
Observational Study
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard treatment involves cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), but recurrences occur in 20-30 per cent of patients. The aim of this study was to define the timing and patterns of recurrence to provide a basis for modifying follow-up of these patients.
METHODS
This observational study examined a prospectively developed multicentre national database (RENAPE working group) to identify patients with recurrence after optimal CRS and HIPEC for PMP. Postoperative complications, long-term outcomes and potential prognostic factors were evaluated.
RESULTS
Of 1411 patients with proven PMP, 948 were identified who had undergone curative CRS and HIPEC. Among these patients, 229 first recurrences (24·2 per cent) were identified: 196 (20·7 per cent) occurred within the first 5 years (early recurrence) and 30 (3·2 per cent) occurred between 5 and 10 years. Three patients developed a first recurrence more than 10 years after the original treatment. The mean(s.d.) time to first recurrence was 2·36(2·21) years. Preoperative chemotherapy and high-grade pathology were significant factors for early recurrence. Overall survival for the entire group was 77·9 and 63·1 per cent at 5 and 10 years respectively. The principal site of recurrence was the peritoneum.
CONCLUSION
Recurrence of PMP was rare after 5 years and exceptional after 10 years.
Topics: Adult; Aged; Combined Modality Therapy; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; Follow-Up Studies; Humans; Hyperthermia, Induced; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Peritoneum; Prognosis; Prospective Studies; Pseudomyxoma Peritonei; Retrospective Studies; Time Factors
PubMed: 30957067
DOI: 10.1002/bjs5.97 -
Journal of Gastrointestinal Oncology Apr 2021Pathology is central to the management of peritoneal surface malignancy. This article highlights some recent advances that have had an impact on patient management or... (Review)
Review
Pathology is central to the management of peritoneal surface malignancy. This article highlights some recent advances that have had an impact on patient management or could do so in the near future. Malignant peritoneal mesothelioma, particularly the epithelioid subtype, is amenable to radical therapy in selected cases, and factors such as ki67 proliferation index, expression of BAP1 and mutation in show promise as prognostic indicators. Our understanding of multicystic mesothelioma has improved in recent years; it is a true neoplasm for which surgery may be indicated. Serous carcinomas involving the peritoneum are now known to originate from tubal epithelium. They are of two distinct types, high grade and low grade, which are now recognized as different neoplasms with distinctive features, oncogenesis and behavior. Pseudomyxoma peritonei (PMP) is an unusual condition that usually arises from an appendiceal mucinous neoplasm. Recent consensus in the classification and nomenclature of these lesions is discussed, including the distinction between low grade and high grade appendiceal mucinous neoplasms (HAMN), and the diagnostic criteria for appendiceal adenocarcinoma. PMP is divided into four prognostic groups: acellular mucin, low grade mucinous carcinoma peritonei, high grade mucinous carcinoma peritonei, and high grade mucinous carcinoma peritonei with signet ring cells. The pseudomyxoma microbiome is a promising area for clinical intervention but has been the subject of little research activity. Goblet cell adenocarcinoma (previously known as 'goblet cell carcinoid') is a distinctive type of appendiceal adenocarcinoma. Its behavior correlates with histologic features, but no general consensus for classification has been reached.
PubMed: 33968439
DOI: 10.21037/jgo-2020-01 -
Journal of Gastrointestinal Oncology Apr 2021High-throughput "-omics" analysis may provide a broader and deeper understanding of cancer biology to define prognostic and predictive biomarkers and identify novel... (Review)
Review
High-throughput "-omics" analysis may provide a broader and deeper understanding of cancer biology to define prognostic and predictive biomarkers and identify novel therapy targets. In this review we provide an overview of studies where the peritoneal tumor component of peritoneal metastases from colorectal cancer (PM-CRC) and pseudomyxoma peritonei (PMP) were analyzed. Most of the available data was derived from DNA mutation analysis, but a brief review of findings from transcriptomic and protein expression analysis was also performed. Studies reporting genomic analysis of peritoneal tumor samples from 1,779 PM-CRC and 623 PMP cases were identified. The most frequently mutated genes in PM-CRC were , , , , and , while in PMP , , , , and mutations were most commonly identified. Analyses were performed by single-gene analyses and to some extent targeted next-generation sequencing, and a very limited amount of broad explorative data exists. The investigated cohorts were typically small and heterogeneous with respect to the methods used and to the reporting of clinical data. This was even more apparent regarding transcriptomic and protein data, as the low number of cases examined and quality of clinical data would not support firm conclusions. Even for the most frequently mutated genes, the results varied greatly; for instance, mutations were reported at frequencies between 20-57% in PM-CRC and 38-100% in PMP. Such variation could be caused by random effects in small cohorts, heterogeneity in patient selection, or sensitivity of applied technology. Although a large number of samples have been subjected to analysis, cross-study comparisons are difficult to perform, and combined with small cohorts and varying quality and detail of clinical information, the observed variation precludes useful interpretation in a clinical context. Although omics data in theory could answer questions to aid management decisions in PM-CRC and PMP, the existing data does not presently support clinical implementation. With the necessary technologies being generally available, the main challenge will be to obtain sufficiently large, representative cohorts with adequate clinical data and standardized reporting of results. Importantly, studies where the focus is specifically on peritoneal disease are needed, where the study designs are aligned with clearly defined research questions to allow robust conclusions. Such studies are highly warranted if patients with PM-CRC and PMP are to derive benefit from recent advances in precision cancer medicine.
PubMed: 33968437
DOI: 10.21037/jgo-20-136 -
Cancers Feb 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of patients with PMP is determined by the degree of cellularity within the mucin (low-grade (LAMN) vs. high-grade (HAMN) histologic features) and by the extent of the disease.
METHODS
Prognostic relevance of tumor markers CA19-9 and CEA, gender, Peritoneal Cancer Index (PCI), and completeness of cytoreduction (CC) after cytoreductive surgery were evaluated on 193 consecutive PMP patients, based on a retrospective analysis of prospectively gathered data from a German tertial referral center.
RESULTS
We demonstrated that low PCI, CC0 status, low-grade histology, and female gender were independent positive prognostic factors for both overall survival (OS) and progression-free survival (PFS). Furthermore, LAMN patients with achieved CC0 status show significantly better OS and PFS compared to those with CC1 status ( = 0.0353 and = 0.0026 respectively). In contrast, the duration and drug of hyperthermic intraperitoneal chemotherapy (HIPEC) were not prognostic in any comparison. Increased CA19-9 and CEA levels were significantly associated with HAMN cases, but also predicted recurrence in patients with low-grade histologies.
CONCLUSION
Our study confirmed the prognostic role of tumor markers and emphasized the importance of CC status and PCI in a large cohort of PMP- and LAMN patients.
PubMed: 36831667
DOI: 10.3390/cancers15041326