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International Journal of Hyperthermia :... Aug 2017Pseudomyxoma peritonei (PMP) is an uncommon disease characterised by mucinous ascites, classically originating from a ruptured low grade mucinous neoplasm of the... (Review)
Review
Pseudomyxoma peritonei (PMP) is an uncommon disease characterised by mucinous ascites, classically originating from a ruptured low grade mucinous neoplasm of the appendix. The natural history of PMP revolves around the "redistribution phenomenon", whereby mucinous tumour cells accumulate at specific sites with relative sparing of the motile small bowel and to a lesser extent other parts of the gastrointestinal tract. Peritoneal tumour accumulates due to gravity and at the sites of peritoneal fluid absorption, namely, the greater and lesser omentum and the under-surface of the diaphragm, particularly on the right. The optimal treatment is complete macroscopic tumour excision termed cytoreductive surgery (CRS) combined with Hyperthermic Intra-Peritoneal Chemotherapy (HIPEC). Total operating time for complete CRS and HIPEC for extensive PMP is around 10 h and generally involves bilateral parietal and diaphragmatic peritonectomies, right hemicolectomy, radical greater omentectomy with splenectomy, cholecystectomy and liver capsulectomy, a pelvic peritonectomy with, or without, rectosigmoid resection and bilateral salpingo-oophorectomy with hysterectomy in females. A unique feature of low grade PMP, which differs from other peritoneal malignancies, includes slow disease progression, which may be asymptomatic until advanced stages. Additionally, very extensive disease with a high "PCI" (Peritoneal Carcinomatosis Index) may still be amenable to complete excision and cure. In cases where complete tumour removal is not feasible, maximum tumour debulking can still result in long-term survival in PMP. PMP is challenging, complex but nevertheless the most rewarding peritoneal malignancy amenable to cure by CRS and HIPEC.
Topics: Female; Humans; Male; Pseudomyxoma Peritonei
PubMed: 28540829
DOI: 10.1080/02656736.2017.1310938 -
Cancers Nov 2021Pseudomyxoma Peritonei (PMP) is an anatomo-clinical condition characterized by the implantation of neoplastic cells on peritoneal surfaces with the production of a large... (Review)
Review
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.
PubMed: 34885075
DOI: 10.3390/cancers13235965 -
Pleura and Peritoneum Mar 2016: The term pseudomyxoma peritonei (PMP) was first described in 1884 and there has been much debate since then over the term. A recent consensus of world experts agreed... (Review)
Review
: The term pseudomyxoma peritonei (PMP) was first described in 1884 and there has been much debate since then over the term. A recent consensus of world experts agreed that PMP should be thought of as a clinical entity characterised by the presence of mucinous ascites, omental cake, peritoneal implants and possibly ovarian involvement. It generally originates from mucinous appendiceal tumours. : This review details the clinical presentation of this unusual condition, presents the new classification system and how this relates to outcome. The pathophysiology of this disease is also explored with a special reference to the relationship of the disease to tumour markers. : A classification system has been agreed upon by the leading experts in PMP which is now divided into low and high grade mucinous carcinomatosis peritonei. This distinction correlates with clinical outcome as does the presence of raised tumour markers preoperatively. : Research needs to be focused on understanding the factors associated with poor prognosis through well designed multi-centred prospective studies. This will allow us to identify patients with bad tumour biology so that targeted treatment based on likely prognosis may then become a reality.
PubMed: 30911604
DOI: 10.1515/pp-2016-0008 -
The Surgical Clinics of North America Jun 2020Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy is an aggressive, potentially curative approach used to treat locoregional disease associated... (Review)
Review
Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy is an aggressive, potentially curative approach used to treat locoregional disease associated with primary and secondary malignancies of the peritoneum. It involves resection of all macroscopic disease larger than 2.5 mm, followed by instillation of hyperthermic chemotherapy directly into the peritoneum for higher drug exposure to microscopic disease. In select patients with primary peritoneal mesothelioma, pseudomyxoma peritonei, colorectal adenocarcinoma, appendiceal adenocarcinoma, or ovarian cancer, with no extra-abdominal metastasis and limited involvement of the peritoneum, the procedure can be performed to increase overall survival.
Topics: Colorectal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermia, Induced; Mesothelioma; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 32402303
DOI: 10.1016/j.suc.2020.02.009 -
European Journal of Surgical Oncology :... Dec 2022Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver... (Review)
Review
BACKGROUND
Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver metastases and lymph node metastases are unusual.
METHODS
From an extensive database, patients with lymph node metastases identified at the time of primary appendiceal cancer resection were selected for special study. The clinical, treatment-related and histologic variables of this group of patients were statistically analyzed for their impact on overall survival.
RESULTS
From a prospectively maintained database of 685 patients with a complete cytoreduction of a mucinous appendiceal neoplasm with peritoneal dissemination, 39 patients (5.6%) had lymph node metastases at the time of primary diagnosis. The median follow-up was 5.0 years and overall median survival was 6.0 years. Histologically, 6 of these patients (15.4%) had an appendiceal mucinous neoplasm - Intermediate type (MACA-Int). In 5 patients, the involved lymph nodes were not within the ileocolic lymph node group. The 7 patients (17.9%) who had a complete or near complete response to neoadjuvant chemotherapy prior to definite cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) showed prolonged survival (HR 4.8 (1.1, 20.5) p = 0.0323). A prior right colon resection required repeat resection in 87% of patients.
CONCLUSION
Long-term survival is unusual but occasionally seen in this group of patients. Response to neoadjuvant chemotherapy is an important determinant of a favorable outcome.
Topics: Humans; Peritoneal Neoplasms; Lymphatic Metastasis; Hyperthermia, Induced; Adenocarcinoma, Mucinous; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Retrospective Studies; Combined Modality Therapy; Survival Rate
PubMed: 35941031
DOI: 10.1016/j.ejso.2022.07.018 -
Journal of B.U.ON. : Official Journal... May 2015Pseudomyxoma peritonei (PMP) is an uncommon clinical condition that typically originates from a perforated epithelial neoplasm of the appendix. The clinical presentation... (Review)
Review
Pseudomyxoma peritonei (PMP) is an uncommon clinical condition that typically originates from a perforated epithelial neoplasm of the appendix. The clinical presentation is variable, often with non-specific symptoms and is associated with abdominal distension in advanced cases. Whilst traditionally considered benign, it is apparent that PMP represents a spectrum of disease and, at best, should be considered a "border-line" malignancy. The condition is characterised by the development of mucinous ascites. Tumour cells and mucin accumulate at characteristic sites within the peritoneal cavity according to the redistribution phenomenon, usually sparing the mobile small bowel. In advanced cases, high volume disease and mucinous ascites lead to compression of the gastrointestinal tract, bowel obstruction, and ultimately, starvation. Controversy still exists over the pathological classification of PMP and its prognostic value. Computed tomography remains the optimal preoperative staging investigation. Elevation of serum tumour markers correlates with a worse prognosis. Optimal treatment involves cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC). With complete cytoreduction and HIPEC an 80% 5 year survival can be achieved in patients with low grade disease. Maximal tumour debulking can produce good palliation and long term survival in a small number of patients. Initial high morbidity and mortality is seen to decrease with increasing experience and this is likely to represent improvement in patient selection and postoperative management as well as surgical expertise.
Topics: Antineoplastic Agents; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 26051332
DOI: No ID Found -
Cleveland Clinic Journal of Medicine Sep 2022
Topics: Humans; Pseudomyxoma Peritonei; Ascites; Peritoneal Neoplasms
PubMed: 37907443
DOI: 10.3949/ccjm.89a.21086 -
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 -
Gastroenterology Clinics of North... Dec 2019"The intestinal transplantation is reserved for patients with life-threatening complications of permanent intestinal failure or underlying gastrointestinal disease. The... (Review)
Review
"The intestinal transplantation is reserved for patients with life-threatening complications of permanent intestinal failure or underlying gastrointestinal disease. The choice of the allograft for a particular patient depends on several factors and the presence of concurrent organ failure, and availability of the donor organs, and specialized care. Combined liver and intestinal transplant allows for patients who have parenteral nutrition-associated liver disease a possibility of improved quality of life and nutrition as well as survival. Intestinal transplantation has made giant strides over the past few decades to the present era where current graft survivals are comparable with other solid organ transplants."
Topics: Abdominal Wall; Allografts; Humans; Intestines; Liver Transplantation; Malabsorption Syndromes; Parenteral Nutrition; Pseudomyxoma Peritonei; Quality of Life; Viscera
PubMed: 31668184
DOI: 10.1016/j.gtc.2019.08.010 -
Orphanet Journal of Rare Diseases Feb 2021Pseudomyxoma 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... (Review)
Review
BACKGROUND
Pseudomyxoma 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 BODY
As 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.
CONCLUSION
Currently, 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.
Topics: Consensus; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33581733
DOI: 10.1186/s13023-021-01723-6