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European Journal of Surgical Oncology :... Jan 2021Pseudomyxoma Peritonei (PMP) is a rare peritoneal malignancy, most commonly originating from a perforated epithelial tumour of the appendix. Given its rarity, randomized... (Review)
Review
Pseudomyxoma Peritonei (PMP) is a rare peritoneal malignancy, most commonly originating from a perforated epithelial tumour of the appendix. Given its rarity, randomized controlled trials on treatment strategies are lacking, nor likely to be performed in the foreseeable future. However, many questions regarding the management of appendiceal tumours, especially when accompanied by PMP, remain unanswered. This consensus statement was initiated by members of the Peritoneal Surface Oncology Group International (PSOGI) Executive Committee as part of a global advisory role in the management of uncommon peritoneal malignancies. The manuscript concerns an overview and analysis of the literature on mucinous appendiceal tumours with, or without, PMP. Recommendations are provided based on three Delphi voting rounds with GRADE-based questions amongst a panel of 80 worldwide PMP experts.
Topics: Appendiceal Neoplasms; Consensus; Delphi Technique; Humans; Neoplasm Grading; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Terminology as Topic
PubMed: 32199769
DOI: 10.1016/j.ejso.2020.02.012 -
Human Pathology Feb 2023Low-grade appendiceal mucinous neoplasms are unique tumors of the appendix, characterized by low-grade mucinous epithelium with villiform, undulating, or flat... (Review)
Review
Low-grade appendiceal mucinous neoplasms are unique tumors of the appendix, characterized by low-grade mucinous epithelium with villiform, undulating, or flat architecture. These tumors lack infiltrative growth or destructive invasion, but can extend into the appendiceal wall by a "pushing" pattern of invasion, with a broad front that can mimic a diverticulum. These neoplasms have a propensity for peritoneal dissemination, resulting in the clinical presentation of pseudomyxoma peritonei. The pathologic staging of these neoplasms is challenging and fraught with confusing terminology and numerous classification systems. This review focuses on the AJCC pathologic staging of these tumors with a focus on challenging situations.
Topics: Humans; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Appendix; Neoplasms, Glandular and Epithelial
PubMed: 35843338
DOI: 10.1016/j.humpath.2022.07.004 -
Cancer Jun 2020The Chicago Consensus Working Group provides multidisciplinary recommendations for the management of appendiceal neoplasms specifically related to the management of...
The Chicago Consensus Working Group provides multidisciplinary recommendations for the management of appendiceal neoplasms specifically related to the management of peritoneal surface malignancies. These guidelines are developed with input from leading experts, including surgical oncologists, medical oncologists, pathologists, radiologists, palliative care physicians, and pharmacists. These guidelines recognize and address the emerging need for increased awareness in the appropriate management of peritoneal surface disease. They are not intended to replace the quest for higher levels of evidence.
Topics: Appendiceal Neoplasms; Chicago; Consensus; Humans; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Practice Guidelines as Topic
PubMed: 32282073
DOI: 10.1002/cncr.32881 -
Klinicka Onkologie : Casopis Ceske a... 2019Pseudomyxoma peritonei is a rare tumorous disease with various grades of malignancy and is characterized by production of mucinous and gelatinous masses. Development of... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei is a rare tumorous disease with various grades of malignancy and is characterized by production of mucinous and gelatinous masses. Development of pseudomyxoma peritonei is usually associated with rupture of appendiceal mucinous tumors and other mucinous tumors of the gastrointestinal tract or ovaries. Pseudomyxoma peritonei is usually divided into three types: low-grade, high-grade, and high-grade with signet ring cells. Staging of the disease is determined by the peritoneal cancer index. Clinical findings are highly variable depending on disease staging. The typical finding of “jelly belly” syndrome worsens with disease progression. The diagnosis is based on the pre-operative cure by imaging methods, especially computed tomography.
METHODS
The Sugarbaker method involves maximal removal of tumorous masses, so-called cytoreductive surgery, and perioperative intraabdominal application of hot cytostatics (hyperthermic intraperitoneal chemotherapy) with the aim of achieving maximal liquidation of tumorous processes.
RESULTS
Our results are comparable with previous published data and confirm high effectivness of this method. The results show statistically very significant extention of overall survival, disease free interval with acceptable lethality 0-12 % and morbidity 27-56%. These results promote this method as the gold standard of treatment of pseudomyxoma peritonei in selected patients.
Topics: Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 31610663
DOI: 10.14735/amko2019329 -
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 -
JAMA Surgery Mar 2021Studies on the prognostic role of hyperthermic intraperitoneal chemotherapy (HIPEC) in pseudomyxoma peritonei (PMP) are currently not available.
IMPORTANCE
Studies on the prognostic role of hyperthermic intraperitoneal chemotherapy (HIPEC) in pseudomyxoma peritonei (PMP) are currently not available.
OBJECTIVES
To evaluate outcomes after cytoreductive surgery (CRS) and HIPEC compared with CRS alone in patients with PMP.
DESIGN, SETTING, AND PARTICIPANTS
This cohort study analyzed data from the Peritoneal Surface Oncology Group International (PSOGI) registry, including 1924 patients with histologically confirmed PMP due to an appendiceal mucinous neoplasm. Eligible patients were treated with CRS with or without HIPEC from February 1, 1993, to December 31, 2017, and had complete information on the main prognostic factors and intraperitoneal treatments. Inverse probability treatment weights based on the propensity score for HIPEC treatment containing the main prognostic factors were applied to all models to balance comparisons between the CRS-HIPEC vs CRS-alone groups in the entire series and in the following subsets: optimal cytoreduction, suboptimal cytoreduction, high- and low-grade histologic findings, and different HIPEC drug regimens. Data were analyzed from March 1 to June 1, 2018.
INTERVENTIONS
HIPEC including oxaliplatin plus combined fluorouracil-leucovorin, cisplatin plus mitomycin, mitomycin, and other oxaliplatin-based regimens.
MAIN OUTCOMES AND MEASURES
Overall survival, severe morbidity (determined using the National Cancer Institute Common Terminology for Adverse Events, version 3.0), return to operating room, and 30- and 90-day mortality. Differences in overall survival were compared using weighted Kaplan-Meier curves, log-rank tests, and Cox proportional hazards multivariable models. A sensitivity analysis was based on the E-value from the results of the main Cox proportional hazards model. Differences in surgical outcomes were compared using weighted multivariable logistic models.
RESULTS
Of the 1924 patients included in the analysis (997 [51.8%] men; median age, 56 [interquartile range extremes (IQRE), 45-65] years), 376 were in the CRS-alone group and 1548 in the CRS-HIPEC group. Patients with CRS alone were older (median age, 60 [IQRE, 48-70] vs 54 [IQRE, 44-63] years), had less lymph node involvement (14 [3.7%] vs 119 [7.7%]), received more preoperative systemic chemotherapy (198 [52.7%] vs 529 [34.2%]), and had higher proportions of high-grade disease (179 [47.6%] vs 492 [31.8%]) and suboptimal cytoreduction residual disease (grade 3, 175 [46.5%] vs 117 [7.6%]). HIPEC was not associated with a higher risk of worse surgical outcomes except with mitomycin, with higher odds of morbidity (1.99; 95% CI, 1.25-3.19; P = .004). HIPEC was associated with a significantly better overall survival in all subsets (adjusted hazard ratios [HRs], 0.60-0.68, with 95% CIs not crossing 1.00). The weighted 5-year overall survival was 57.8% (95% CI, 50.8%-65.7%) vs 46.2% (95% CI, 40.3%-52.8%) for CRS-HIPEC and CRS alone, respectively (weighted HR, 0.65; 95% CI, 0.50-0.83; P < .001; E-value, 2.03). Such prognostic advantage was associated with oxaliplatin plus fluorouracil-leucovorin (HR, 0.42; 95% CI, 0.19-0.93; P = .03) and cisplatin plus mitomycin (HR, 0.57; 95% CI, 0.42-0.78; P = .001) schedules.
CONCLUSIONS AND RELEVANCE
In this cohort study, HIPEC was associated with better overall survival when performed after CRS in PMP, generally without adverse effects on surgical outcomes.
Topics: Adult; Aged; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Appendiceal Neoplasms; Cohort Studies; Cytoreduction Surgical Procedures; Drug Administration Schedule; Female; Humans; Hyperthermic Intraperitoneal Chemotherapy; Male; Middle Aged; Proportional Hazards Models; Pseudomyxoma Peritonei; Survival Rate; Treatment Outcome
PubMed: 33502455
DOI: 10.1001/jamasurg.2020.6363 -
Cancers Feb 2023Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is a novel intraperitoneal drug delivery method of low-dose chemotherapy as a pressurized aerosol in patients... (Review)
Review
BACKGROUND
Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is a novel intraperitoneal drug delivery method of low-dose chemotherapy as a pressurized aerosol in patients affected by peritoneal cancer of primary or secondary origin. We performed a systematic review and meta-analysis with the aim of assessing the feasibility, safety, and efficacy of PIPAC.
METHODS
A systematic literature search was performed using Medline and Web of Science databases from 1 January 2011, to inception, to 31 December 2021. Data were independently extracted by two authors. The Newcastle-Ottawa Scale was used to assess the quality and risk of bias of studies. Meta-analysis was performed for pathological response, radiological response, PCI variation along treatment, and for patients undergoing three or more PIPAC. Pooled analyses were performed using the Freeman-Tukey double arcsine transformation, and 95% CIs were calculated using Clopper-Pearson exact CIs in all instances.
RESULTS
A total of 414 papers on PIPAC were identified, and 53 studies considering 4719 PIPAC procedure in 1990 patients were included for analysis. The non-access rate or inability to perform PIPAC pooled rate was 4% of the procedures performed. The overall proportion of patients who completed 3 or more cycles of PIPAC was 39%. Severe toxicities considering CTCAE 3-4 were 4% (0% to 38.5%). In total, 50 studies evaluated deaths within the first 30 postoperative days. In the included 1936 patients were registered 26 deaths (1.3%). The pooled analysis of all the studies reporting a pathological response was 68% (95% CI 0.61-0.73), with an acceptable heterogeneity (I 28.41%, = 0.09). In total, 10 papers reported data regarding the radiological response, with high heterogeneity and a weighted means of 15% (0% to 77.8%). PCI variation along PIPAC cycles were reported in 14 studies. PCI diminished, increased, or remained stable in eight, one and five studies, respectively, with high heterogeneity at pooled analysis. Regarding survival, there was high heterogeneity. The 12-month estimated survival from first PIPAC for colorectal cancer, gastric cancer, gynecological cancer and hepatobiliary/pancreatic cancer were, respectively, 53%, 25%, 59% and 37%.
CONCLUSIONS
PIPAC may be a useful treatment option for selected patients with PM, with acceptable grade 3 and 4 toxicity and promising survival benefit. Meta-analysis showed high heterogeneity of data among up-to-date available studies. In a subset analysis per primary tumor origin, pathological tumor regression was documented in 68% of the studies with acceptable heterogeneity. Pathological regression seems, therefore, a reliable outcome for PIPAC activity and a potential surrogate endpoint of treatment response. We recommend uniform selection criteria for patients entering a PIPAC program and highlight the urgent need to standardize items for PIPAC reports and datasets.
PubMed: 36831468
DOI: 10.3390/cancers15041125 -
Surgical Oncology Mar 2022Cytoreductive surgery and intraperitoneal chemotherapy may offer chance for cure for patients with peritoneal metastasis. Many variations emerged, causing uncertainty... (Review)
Review
Cytoreductive surgery and intraperitoneal chemotherapy may offer chance for cure for patients with peritoneal metastasis. Many variations emerged, causing uncertainty when choosing the most suitable variant. By reviewing variability encountered in the management of peritoneal metastasis, we aim to raise awareness about this issue and hopefully initiate efforts to solve it. We review variance encountered in all aspects of this complex field of surgical oncology, indications, patient selection criteria, definition and extent of cytoreductive surgery and the numerous variables of intraperitoneal chemotherapy. Best benefit was achieved with pseudomyxoma peritonei, and to lesser extent in colorectal, ovarian and gastric cancer, but Indications keep expanding to include other tumors pathologies. Selection of patients depends on numerous prognostic indicators and criteria, according to tumor extent and pathology. The standard definition of cytoreductive surgery remains the same, but the boundaries of resection expand. Numerous chemotherapy regimens and administration methods are used, in search for best possible benefit. This variance must be reduced, to make the best use of, and further spread this treatment combination. Practical simple guidelines are needed for surgical oncologists willing to utilize this treatment for their patients, to be considered a true standard of care.
Topics: Cytoreduction Surgical Procedures; Humans; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms
PubMed: 34875459
DOI: 10.1016/j.suronc.2021.101676