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International Journal of Molecular... Nov 2020The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood.... (Review)
Review
The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood. Epithelial-mesenchymal transition (EMT) plays a role in cancer development and progression by converting static epithelial cells into the migratory and microenvironment-interacting mesenchymal cells, and by the modulation of chemoresistance and stemness of tumor cells. Several findings highlight that pathways involved in EMT and its reverse process (mesenchymal-epithelial transition, MET), now collectively called epithelial-mesenchymal plasticity (EMP), play a role in peritoneal metastases. So far, the relevance of factors linked to EMP in a unique peritoneal malignancy such as pseudomyxoma peritonei (PMP) has not been fully elucidated. In this review, we focus on the role of epithelial-mesenchymal dynamics in the metastatic process involving mucinous neoplastic dissemination in the peritoneum. In particular, we discuss the role of expression profiles and phenotypic transitions found in PMP in light of the recent concept of EMP. A better understanding of EMP-associated mechanisms driving peritoneal metastasis will help to provide a more targeted approach for PMP patients selected for locoregional interventions involving cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Topics: Animals; Biomarkers; Cell Plasticity; Disease Management; Disease Susceptibility; Epithelial-Mesenchymal Transition; Humans; Osteogenesis; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33266161
DOI: 10.3390/ijms21239120 -
Health Technology Assessment... Feb 2004This systematic review examines the clinical and cost-effectiveness of the Sugarbaker procedure for treating pseudomyxoma peritonei (PMP) and the costs of the procedure... (Review)
Review
OBJECTIVES
This systematic review examines the clinical and cost-effectiveness of the Sugarbaker procedure for treating pseudomyxoma peritonei (PMP) and the costs of the procedure in the UK.
DATA SOURCES
Electronic databases, bibliographies of related papers and experts in the field were used as sources for English language studies available up to September 2002.
REVIEW METHODS
Evidence of the clinical effectiveness of the Sugarbaker procedure for PMP was synthesised through a narrative review with full tabulation of results of all included studies. The economic modelling used a Monte-Carlo simulation model populated with UK price data to estimate likely UK costs.
RESULTS
Five retrospective case-series reports assessing the Sugarbaker procedure met the inclusion criteria for the review, although they were found to be of poor quality when judged against standard criteria for assessing methodological standard. There appears to be some benefit for people with PMP who undergo treatment with the Sugarbaker procedure. Commonly reported complications of the Sugarbaker procedure were anastomotic leaks, fistula formation, wound infection, small bowel perforations/obstructions and pancreatitis. One costing study of poor methodological quality and set in the USA was found. This study, together with UK unit price data and expert advice, was used to populate a Monte-Carlo simulation model to estimate the marginal cost of operating a service to provide treatment for PMP using the Sugarbaker technique rather than standard treatment. The results of the Monte-Carlo simulation model showed that the cost for one patient over a maximum of 5 years would be about 9700 British pounds, with a standard deviation of about 1300 British pounds (although costs incurred in setting up the specific service or training the staff were not included). The US study showed a ten-fold higher cost. The Monte-Carlo analysis showed that the variation around the mean was not very high. The most likely factor influencing the variation of the costs was the length of procedure. No sensitivity analysis could be done of the alternative treatment.
CONCLUSIONS
The economic results should be seen as merely an example of the likely marginal costs of the Sugarbaker procedure, as more information about the current alternative is required. Trained and experienced staff are required to implement the procedure and inevitably time and cost will be involved in developing the appropriate teams. Although the procedure requires some specialist equipment and maintenance, such as smoke evacuators, these should have limited effect on setting up the service. PMP is a relatively rare condition with approximately 50 new cases per year in the UK and the impact of an increase in the demand for services should be limited. Evidence is needed for the effectiveness of maximal cytoreductive surgery compared with surgical debulking, using different intraoperative intraperitoneal chemotherapy strategies, and for the effectiveness of treatments in patients who have residual disease following maximal efforts at cytoreduction. Further research involving high-quality prospective cohort studies with economic evaluations would be valuable.
Topics: Adult; Aged; Aged, 80 and over; Costs and Cost Analysis; Female; Humans; Male; Middle Aged; Pseudomyxoma Peritonei; State Medicine; Surgical Procedures, Operative; Treatment Outcome; United Kingdom
PubMed: 14960255
DOI: 10.3310/hta8070 -
Chinese Clinical Oncology Jun 2021Pseudomyxoma peritonei is a rare disease with a reported prevalence of about 1-3 per million people annually. Cytoreductive surgery and perioperative hyperthermic...
Pseudomyxoma peritonei is a rare disease with a reported prevalence of about 1-3 per million people annually. Cytoreductive surgery and perioperative hyperthermic intraperitoneal chemotherapy are considered as treatment options improving disease control or long-term survival. However, for patients with incomplete cytoreduction or debulking surgery, outcomes are significantly poorer compared with patients who have obtained complete or optimal cytoreduction. In cases of high-grade pseudomyxoma peritonei that are considered inoperable and/or unresectable, combination chemotherapy regimen with a neo-angiogenesis inhibitor such as bevacizumab is recommended. In this report, a 62-year-old Japanese man presented with abdominal distention. Examination of ascites demonstrated a jelly-like consistency and peritoneal pseudomyxoma was suspected. To relieve progressive symptoms, palliative debulking surgery with total colectomy was performed. Postoperative pathology confirmed high-grade appendiceal mucinous neoplasm with high-grade pseudomyxoma peritonei. In our case, combination chemotherapy with trifluridine/tipiracil (TAS-102) and bevacizumab was initiated after palliative debulking surgery. As a result, carcinoembryonic antigen level was kept stable and the volume of ascites remained almost the same as at the beginning of treatment for more than 2 years. In conclusion, combination chemotherapy comprising TAS-102 and bevacizumab in patients with palliative debulking could be a useful option for patients with high-grade mucinous appendiceal neoplasm and high-grade pseudomyxoma peritonei.
Topics: Bevacizumab; Cytoreduction Surgical Procedures; Drug Combinations; Humans; Male; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Pyrrolidines; Thymine; Trifluridine
PubMed: 33752412
DOI: 10.21037/cco-21-2 -
Current Oncology (Toronto, Ont.) Jun 2016Peritoneal carcinomatosis (ptc) represents advanced malignant disease and has generally been associated with a grim prognosis. Peritoneal surface malignancy is often the... (Review)
Review
Peritoneal carcinomatosis (ptc) represents advanced malignant disease and has generally been associated with a grim prognosis. Peritoneal surface malignancy is often the major source of morbidity and mortality; it is of major concern in cancer management. Although ptc is categorized as metastatic disease, it represents a special disease pattern considered to be a locoregional disease limited to the abdominal cavity. The combination of cytoreductive surgery (crs) and intraoperative hyperthermic intraperitoneal chemotherapy (hipec) has successfully been used as locoregional treatment for selected patients with ptc from gastric, colorectal, and ovarian cancer; with mesothelioma; and with pseudomyxoma peritonei. In the prophylactic setting, hipec can also be used to prevent ptc in high-risk patients, and the first results of the "second-look" approach are promising. Patient selection-in which the risks of perioperative morbidity and mortality, which are analogous to those for any other major gastrointestinal surgery, are assessed-is of utmost importance. Those risks have to be weighed against the anticipated survival benefit, which depends mainly on tumour biology, extent of disease, and probability of achieving complete crs. The present review discusses the principles of crs and hipec, the most significant recent clinical data, and current perspectives concerning the application of this treatment modality in various malignancies. Ongoing trials and future directions are noted. It appears that the combination of crs and hipec is an indispensable tool in the oncologist's armamentarium.
PubMed: 27330364
DOI: 10.3747/co.23.2831 -
Annals of Surgery May 2023To report our experience with the combination of radical surgical excision and intestinal transplantation in patients with recurrent pseudomyxoma peritonei (PMP) not...
OBJECTIVE
To report our experience with the combination of radical surgical excision and intestinal transplantation in patients with recurrent pseudomyxoma peritonei (PMP) not amenable to further cytoreductive surgery (CRS).
BACKGROUND
CRS and heated intraoperative peritoneal chemotherapy are effective treatments for many patients with PMP. In patients with extensive small bowel involvement or nonresectable recurrence, disease progression results in small bowel obstruction, nutritional failure, and fistulation, with resulting abdominal wall failure.
METHODS
Between 2013 and 2022, patients with PMP who had a nutritional failure and were not suitable for further CRS underwent radical debulking and intestinal transplantation at our centre.
RESULTS
Fifteen patients underwent radical exenteration of affected intra-abdominal organs and transplantation adapted according to the individual case. Eight patients had isolated small bowel transplantation and 7 patients underwent modified multivisceral transplantation. In addition, in 7 patients with significant abdominal wall tumor involvement, a full-thickness vascularized abdominal wall transplant was performed. Two of the 15 patients died within 90 days due to surgically related complications. Actuarial 1-year and 5-year patient survivals were 79% and 55%, respectively. The majority of the patients had significant improvement in quality of life after transplantation. Progression/recurrence of disease was detected in 91% of patients followed up for more than 6 months.
CONCLUSION
Intestinal/multivisceral transplantation enables a more radical approach to the management of PMP than can be achieved with conventional surgical methods and is suitable for patients for whom there is no conventional surgical option. This complex surgical intervention requires the combined skills of both peritoneal malignancy and transplant teams.
Topics: Humans; Pseudomyxoma Peritonei; Follow-Up Studies; Quality of Life; Peritoneal Neoplasms; Peritoneum; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Retrospective Studies; Combined Modality Therapy
PubMed: 36468404
DOI: 10.1097/SLA.0000000000005769 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Aug 2021Pseudomyxoma peritonei (PMP) is a rare low-grade malignant tumor, which is difficult to operate with many postoperative complications. In recent years, enhanced recovery...
OBJECTIVES
Pseudomyxoma peritonei (PMP) is a rare low-grade malignant tumor, which is difficult to operate with many postoperative complications. In recent years, enhanced recovery after surgery (ERAS) has been greatly developed in the perioperative management of surgical diseases, and it plays an important role in improving the postoperative prognosis of surgical patients. This study was conducted to explore the application of ERAS in the perioperative management of PMP patients, and to study the effect of ERAS on postoperative respiratory and digestive tract complications.
METHODS
We retrospectively analyzed clinical data of patients with PMP from January 2014 to December 2018. These patients were treated with surgery in our center and they were divided into an observation group and a control group. The patients in the control group didn't perform ERAS in perioperative period, and patients in the observation group was performed ERAS. Then, we analyzed and compared the postoperative pulmonary complications (PPC) and gastrointestinal function between the 2 groups.
RESULTS
There was no significant difference in the incidence of atelectasis, pleural effusion, pulmonary infection and acute respiratory distress syndrome (ARDS) between the two groups, but the total incidence of PPC in the observation group was significantly lower than that in the control group (=0.032). The incidence of postoperative gastrointestinal dysfunction (PGID) in the observation group was significantly lower than that in the control group (=0.025), and the postoperative first exhaust time, first defecation time, oral feeding time, and albumin level in the observation group were all better than those in the control group (all <0.05).
CONCLUSIONS
ERAS can significantly reduce the incidence of postoperative PPC and PGID in the PMP patients and improve their postoperative recovery.
Topics: Enhanced Recovery After Surgery; Humans; Length of Stay; Lung; Peritoneal Neoplasms; Postoperative Complications; Pseudomyxoma Peritonei; Recovery of Function; Retrospective Studies
PubMed: 34565727
DOI: 10.11817/j.issn.1672-7347.2021.200646 -
ANZ Journal of Surgery Dec 2022
Topics: Humans; Pseudomyxoma Peritonei; Appendix; Appendiceal Neoplasms; Peritoneal Neoplasms
PubMed: 36527695
DOI: 10.1111/ans.17971 -
Journal of Surgical Case Reports May 2023Appendiceal collision tumours are extremely rare, with most reported cases describing tumours consisting of a mucinous component and a neuroendocrine component....
Appendiceal collision tumours are extremely rare, with most reported cases describing tumours consisting of a mucinous component and a neuroendocrine component. Low-grade appendiceal mucinous neoplasms, in some cases, have a tendency to rupture and disseminate their mucin-producing cells throughout the abdominal cavity, leading to a clinical syndrome known as pseudomyxoma peritonei (PMP). We present the case of a 64-year-old male who initially presented with acute appendicitis and was subsequently found to have PMP and appendiceal malignancy. After several years of scans, surgical intervention and histological analysis, it became apparent that the appendiceal malignancy was comprised of distinct cell types. The patient underwent two rounds of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, which resulted in a 2-year disease-free period. Unfortunately, the PMP recurred, having morphological changes consistent with a more aggressive disease process.
PubMed: 37251256
DOI: 10.1093/jscr/rjad281 -
Frontiers in Oncology 2021This study aimed to investigate the value of using ultrasound (US) preoperatively for predicting pathological classification, complete cytoreduction possibility, and...
OBJECTIVES
This study aimed to investigate the value of using ultrasound (US) preoperatively for predicting pathological classification, complete cytoreduction possibility, and survival rate of patients with pseudomyxoma peritonei (PMP).
METHODS
We retrospectively studied PMP patients who were scheduled for cytoreductive surgery between May 2009 and October 2019. US examination was performed before surgery. Factors related to high-grade pathology and poor completeness of cytoreduction (CC) score were identified. Associations between ultrasound characteristics and the survival status were also examined to identify independent predictive factors.
RESULTS
PMP patients with clear ascites, abdominal lymph nodes, omental cake, abdominal mass, portal infiltration, and mesenteric involvement visible on US were considered to have high-grade pathology. Various US features were shown to be independent prognostic markers for inadequate cytoreduction in PMP patients. Portal infiltration and mesenteric involvement were significant prognostic factors for lower survival rates (hazard ratio = 3.092, 3.932, respectively). A visual nomogram including these factors was constructed to predict survival rates. The consistency index was 0.777, which reflected relatively high accuracy.
CONCLUSIONS
Preoperative US has the potential to predict pathological grade and resectability of PMP. Portal infiltration and mesenteric involvement were independent predictors of poor clinical outcomes in PMP patients. Furthermore, a simple-to-use nomogram derived from our study data may be a helpful visual tool in clinical practice to predict 1-, 2-, and 3-year survival rates for PMP patients.
PubMed: 34604030
DOI: 10.3389/fonc.2021.690178 -
International Journal of Hyperthermia :... May 2018Radiological imaging often underestimates the extent of low volume peritoneal disease. The benefit of laparoscopy in assessing peritoneal metastases from colorectal and... (Review)
Review
INTRODUCTION
Radiological imaging often underestimates the extent of low volume peritoneal disease. The benefit of laparoscopy in assessing peritoneal metastases from colorectal and gastric cancer is accepted, but is inconclusive for appendiceal malignancy. We report our experience of diagnostic (DL) and therapeutic laparoscopy (TL) in patients with appendiceal tumours to determine indications and role in assessment and management.
METHODS
A retrospective review of a National Peritoneal Malignancy Centre's prospectively maintained database was performed. All patients with appendiceal neoplasms who underwent DL or TL between September 2011 and January 2016 were included. The indications and outcomes of the laparoscopy, complications and interval to laparotomy were evaluated.
RESULTS
Six hundred and eighty-five patients underwent surgery for appendiceal neoplasms during the study period, of which 73 (10.6%) underwent laparoscopy (50 DL, 23 TL). The main indications for DL were to clarify imaging and stage patients with high-risk histology. Indications for TL were an abnormal appendix without gross pseudomyxoma peritonei (PMP) or with low volume PMP, and concerns for fertility in the presence of PMP. DL resulted in 16 patients (32%) avoiding laparotomy because of extensive disease or no tumour found. Overall, 28 patients were assessed to have resectable disease and at laparotomy, 25/28 had complete cytoreduction with three patients unresectable. In the TL group, appendicectomy and peritoneal lavage was achieved in all four women with fertility concerns, allowing them to conceive thereafter. There were no complications.
CONCLUSIONS
Patients with high-risk appendiceal neoplasm may benefit from DL, and potentially avoid unnecessary laparotomy. TL is useful in patients with low volume PMP and may aid fertility in selected patients.
Topics: Adult; Aged; Appendiceal Neoplasms; Female; Humans; Laparoscopy; Male; Middle Aged; Prospective Studies; Retrospective Studies
PubMed: 28605985
DOI: 10.1080/02656736.2017.1338363