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Human Pathology Mar 2020Pseudomyxoma peritonei (PMP) is an extremely rare malignancy, characterized by extensive peritoneal implantation and colloidal ascites. This study was to explore the... (Comparative Study)
Comparative Study
BACKGROUND
Pseudomyxoma peritonei (PMP) is an extremely rare malignancy, characterized by extensive peritoneal implantation and colloidal ascites. This study was to explore the pathological prognostic factors of PMP.
METHODS
Specimens from 155 PMP patients were analyzed by H&E and immunohistochemistry. Parameters included primary tumor location, histological grade, lymph node metastasis, tumor emboli in the blood and lymph vessels, perineural invasion, Ki67 labeling index, p53, mismatch repair (MMR) gene mutations, MUC1, MUC2, MUC5AC, and MUC6. Clinicopathological and follow-up data were subjected to univariate and multivariate analyses.
RESULTS
The patients included 63.2% (n = 98) low-grade mucinous carcinoma peritonei, 31.6% (n = 49) high-grade mucinous carcinoma peritonei and 5.2% (n = 8) high-grade mucinous carcinoma peritonei with signet ring cells. There were 9.7% (n = 15) with lymph node metastasis; 11.6% (n = 18) with angiolymphatic invasion; 6.3% (n = 8) with defective MMR (dMMR); 35.5% (n = 55) with Ki67 labeling index ≥ 50%; 36.1% (n = 56) with p53 mutation. For PMP from appendiceal origin (n = 140), univariate analysis identified 10 potential prognostic factors. But Multivariate analysis identified only histologic grade was the independent prognostic factor for OS. Mortality risk of high-grade peritoneal mucinous carcinoma or high-grade peritoneal mucinous carcinoma with signet ring cells was 7.056 times (P < .0001, 95% CI: 2.701-18.435) or 27.224 times (P < .0001, 95% CI: 6.207-119.408), respectively, higher than low-grade.
CONCLUSIONS
For PMP from the appendiceal origin, histological grade could be the only independent prognostic factor.
Topics: Adult; Aged; Biomarkers, Tumor; DNA Mismatch Repair; Female; Humans; Lymphatic Metastasis; Male; Middle Aged; Mutation; Neoplasm Grading; Neoplastic Cells, Circulating; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Risk Assessment; Risk Factors
PubMed: 31926211
DOI: 10.1016/j.humpath.2019.12.008 -
Annals of Gastroenterological Surgery Jul 2024Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is established in the management of pseudomyxoma peritonei (PMP), selected...
Long-term outcomes and survival analysis of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy in patients with pseudomyxoma peritonei at a newly established peritoneal malignancy centre in Japan.
BACKGROUND
Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is established in the management of pseudomyxoma peritonei (PMP), selected cases of peritoneal mesothelioma, and resectable colorectal or ovarian peritoneal metastases in Western countries. However, the efficacy and feasibility of these techniques are not well established in the Asian population, and little has been reported on long-term survival outcomes for surgically resected PMP patients.
MATERIALS AND METHODS
Retrospective analysis of a prospective database of short- and longer-term outcomes of consecutive patients who underwent CRS and HIPEC for PMP in a newly established peritoneal malignancy unit in Japan between 2010 and 2016.
RESULTS
A total of 105 patients underwent CRS and HIPEC and 57 maximal tumor debulking (MTD) for pseudomyxoma peritonei. In the CRS group, the primary tumor was appendiceal in 94 patients (90%) followed by ovarian and colorectal. Major postoperative complications occurred in 22/105 patients (21%) with one in-hospital mortality (0.9%). The 5-year overall and disease-free survival rates for the CRS group were 74.2% and 50.1%, respectively. Multivariate analysis revealed unfavorable histology to be the significant predictor of reduced overall and disease-free survival. Completeness of cytoreduction, CA19-9, and CA125 were also associated with disease-free survival.
CONCLUSIONS
This is the first report on long-term outcomes and survival analysis of CRS and HIPEC for PMP in the Asian population. CRS and HIPEC can be conducted with reasonable safety and favorable survival in a new center. Complete tumor removal and histological type are the strongest prognostic factors for both overall and disease-free survival.
PubMed: 38957568
DOI: 10.1002/ags3.12791 -
Cancers Sep 2022Pseudomyxoma peritonei (PMP) is ideally treated by cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), leading to significant morbidity....
Pseudomyxoma peritonei (PMP) is ideally treated by cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), leading to significant morbidity. Beyond the histologic grade, the prognosis lies in the completeness of cytoreduction (CC-score of 0/1 vs. 2/3) and the severe complication rate. The mucinous nature of the peritoneal implants sometimes induces liver and/or spleen scalloping on imaging. The predictive value of scalloping was assessed regarding resectability, grade, survival and severe morbidity. This monocentric, retrospective analysis compared CC-0/1 with CC-2/3 groups regarding liver and spleen scalloping parameters, assessed on pre-operative computed tomography (CT) scan, reviewed for the study. In addition, prognostic factors of severe complications and recurrence-free and overall survivals were explored in the CC-0/1 population. Overall, 129 patients were included (109 CC-0/1, 20 CC-2/3), with 58 (45%) exhibiting scalloping. All patients with splenic scalloping also had a liver one. Scalloping was more frequent (75% vs. 39%), with greater median maximal depth (21 vs. 11 mm) and higher PCI (32 vs. 14) in the CC-2/3 population, but was not predictive of either grade or survival. In CC-0/1 patients, survivals and postoperative complications were not affected by scalloping parameters. Scalloping appeared as a marker of advanced PMP, but was not predictive of grade, severe complications, or long-term outcomes.
PubMed: 36139595
DOI: 10.3390/cancers14184434 -
Insights Into Imaging Jul 2023Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address... (Review)
Review
Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address their differential diagnosis, staging and treatment. In this article, we explain the pathophysiology of these processes and lay out the role of different imaging techniques in their evaluation. Then, we review the clinical and epidemiological aspects, the main radiological features and the therapeutic approaches for each primary and secondary peritoneal neoplasm, with surgical and pathological correlation. We further describe other rare peritoneal tumors of uncertain origin and a variety of entities that may mimic peritoneal malignancy. Finally, we summarize the key imaging findings of each peritoneal neoplasm to facilitate an accurate differential diagnosis that may impact patient management.Clinical relevance statementImaging plays an essential role in the evaluation of peritoneal malignancies, assessing their extension, detecting unfavorable sites of involvement and facilitating an accurate differential diagnosis, helping to choose the best therapeutic approach.
PubMed: 37395913
DOI: 10.1186/s13244-023-01417-6 -
Clinics in Colon and Rectal Surgery Nov 2020Peritoneal metastasis from colorectal cancer (PM-CRC) is used to be considered a systemic and fatal condition; however, it has been growingly accepted that PM-CRC can... (Review)
Review
Peritoneal metastasis from colorectal cancer (PM-CRC) is used to be considered a systemic and fatal condition; however, it has been growingly accepted that PM-CRC can still be local disease rather than systemic disease as analogous to liver or lung metastasis. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is now considered an optimal treatment for PM-CRC with accumulating evidence. There is a good reason that CRS + HIPEC, widely accepted as a standard of care for pseudomyxoma peritonei (PMP), could be a viable option for PM-CRC given a similarity between PM-CRC and PMP. Recent years have also seen that modern systemic chemotherapy with or without molecular targeted agents can be effective for PM-CRC. It is possible that neoadjuvant or adjuvant chemotherapy combined with CRS + HIPEC could further improve outcomes. Patient selection, utilizing modern images and increasingly laparoscopy, is crucial. Particularly, diagnostic laparoscopy is likely to play a significant role in predicting the likelihood of achieving complete cytoreduction and assessing the peritoneal cancer index score.
PubMed: 33162842
DOI: 10.1055/s-0040-1714242 -
Innovative Surgical Sciences Mar 2024Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of... (Review)
Review
Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of affected patients' prognoses. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are associated with favorable long-term outcomes in carefully selected patients with peritoneal metastases (PM). We aim to summarize the current results about the initial malignancies and their peritoneal spreads. The current literature has been scrutinized, and studies between 2016 and 2022 were included wherein long-term, progression-free (PFS), and overall survival (OS) data were considered relevant information. Medline, Embase, and Google Scholar have been the main sources. Hereby, we cover all the primer malignancies: gastric, ovarian, and colorectal cancers with peritoneal metastases (PM), malignant peritoneal mesothelioma, and pseudomyxoma peritonei. Examining the advances in the current peer-reviewed literature about the indications of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), target groups, risk factors, and other influencing elements, we intend to provide a complex state-of-the-art report, establishing the relevant aspects of that emerging treatment method.
PubMed: 38826635
DOI: 10.1515/iss-2023-0055 -
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 -
Medicine Jul 2020The aim of study was to develop and validate nomograms for predicting overall survival (OS) and cancer-specific survival (CSS) of patients with pseudomyxoma peritonei...
BACKGROUND
The aim of study was to develop and validate nomograms for predicting overall survival (OS) and cancer-specific survival (CSS) of patients with pseudomyxoma peritonei (PMP) and compare the predictive accuracy with the American Joint Committee on Cancer (AJCC) staging system.
METHODS
Data of 4959 PMP patients who underwent surgical resection were collected between 2004 and 2015 from the Surveillance Epidemiology and End Results (SEER) database. All included patients were divided into training (n = 3307) and validation (n = 1652) cohorts. The Kaplan-Meier method and Cox proportional hazard model were applied. Nomograms were validated by discrimination and calibration. Finally, concordance index (C-index) was used to compare the predictive performance of nomograms with that of the AJCC staging system.
RESULTS
According to the univariate and multivariate analyses of training sets, both nomograms for predicting OS and CSS combining age, grade, location, N stage, M stage, and chemotherapy were identified. Nomograms predicting OS also incorporated T stage and the number of lymph nodes removed (LNR). The calibration curves showed good consistency between predicted and actual observed survival. Moreover, C-index values demonstrated that the nomograms predicting both OS and CSS were superior to the AJCC staging system in both cohorts.
CONCLUSION
We successfully developed and validated prognostic nomograms for predicting OS and CSS in PMP patients. Two nomograms were more accurate and applicable than the AJCC staging system for predicting patient survival, which may help clinicians stratify patients into different risk groups, tailor individualized treatment, and accurately predict patient survival in PMP.
Topics: Adult; Aged; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Staging; Nomograms; Peritoneal Neoplasms; Prognosis; Progression-Free Survival; Proportional Hazards Models; Pseudomyxoma Peritonei; Reproducibility of Results; SEER Program; Survival Analysis; Young Adult
PubMed: 32756083
DOI: 10.1097/MD.0000000000020963 -
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 -
Gastroenterology Research and Practice 2020Pseudomyxoma peritonei (PMP) is a rare neoplasm involving the peritoneum. Most PMPs are low-grade appendicular mucinous neoplasms (LAMNs). There have been no reports of...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare neoplasm involving the peritoneum. Most PMPs are low-grade appendicular mucinous neoplasms (LAMNs). There have been no reports of PMP originating from a transverse colonic mucinous adenocarcinoma and causing metastatic mucinous adenocarcinoma. . We report a 46-year-old woman who presented with a right abdominal mass of more than 4-month duration. Transverse colonic mucinous adenocarcinoma, PMP, and ovarian metastatic mucinous adenocarcinoma were diagnosed. The patient's diet was normal, and she had no abdominal pain or bloating. The abdomen mass increased in the month before treatment. After chemotherapy, the transverse colon mass and ovarian giant cyst were resected and about 2000 mL of gelatinous tumor tissue was removed. Postoperative histology confirmed PMP from the transverse colonic mucinous adenocarcinoma, ovarian metastatic mucinous adenocarcinoma, and mesocolon metastatic cancer. Multiple lung metastases appeared 8 months after surgery. The patient died 29 months after surgery because of an inability to eat and poor nutrition. A systematic literature review of the management and outcome of all known similar cases is also presented.
CONCLUSIONS
This is the first report of PMP originating from a transverse colonic mucinous adenocarcinoma. It was diagnosed during resective surgery, involved ovarian metastasis, and survival was short. We did an extensive literature review in order to describe the clinical characteristics, histopathological findings, genetic profile, and potential treatments of PMP caused by nonappendiceal mucinous adenocarcinoma.
PubMed: 32714388
DOI: 10.1155/2020/5826214