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Scandinavian Journal of Surgery : SJS :... Jun 2024Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to...
BACKGROUND AND AIMS
Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to investigate the effect of disease recurrence on overall survival in patients with PMP after cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC).
METHODS
One-hundred thirty-two consecutive PMP patients treated with CRS + HIPEC at Helsinki University Hospital between 2008 and 2017 were included. The impact of clinicopathological and treatment-related characteristics on recurrence and overall survival was evaluated.
RESULTS
The median follow-up time in the study was 5.04 (range = 0.05-11.60) years. In 121 (91.7%) patients, the disease was classified as low grade and 11 (8.3%) had high-grade disease. In the low-grade group, 26 (21.5%) patients developed a recurrence during follow-up compared to 6 (54.5%) patients in the high-grade group. In the low-grade group, cumulative survival was 98.2%, 91.4%, and 91.4% at 3, 6, and 8 years, respectively. In the high-grade group, cumulative survival was 90.0% and 78.8% at 3 and 6 years, respectively. In patients with recurrent disease, the cumulative survival was 100%, 84.6%, and 84.6% at 3, 6, and 8 years in the low-grade category and 80.0% and 60.0% at 3 and 6 years in the high-grade category, respectively. In the low-grade group, a statistically significant correlation with recurrence but not with overall survival was identified with peritoneal cancer index (PCI), carcinoembryonic antigen (CEA), and the number of affected regions.
CONCLUSION
The recurrence of low-grade PMP does not significantly affect overall survival of patients. Disease extent may not be a prognostic indicator after curative CRS and HIPEC in low-grade PMP.
Topics: Humans; Pseudomyxoma Peritonei; Cytoreduction Surgical Procedures; Female; Male; Middle Aged; Retrospective Studies; Peritoneal Neoplasms; Hyperthermic Intraperitoneal Chemotherapy; Aged; Neoplasm Recurrence, Local; Adult; Combined Modality Therapy; Survival Rate; Finland
PubMed: 37828760
DOI: 10.1177/14574969231200653 -
World Journal of Gastrointestinal... Sep 2019Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term. The word pseudomyxoma derives from pseudomucin, a type of mucin. PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary, a term that has disappeared from today's classifications of cystic ovarian neoplasms. It is known today that in the majority of cases, the origin for PMP is an appendiceal neoplasm, often of low histological grade. Currently, ovarian tumors are wrongly being considered a significant recognized etiology of PMP. PMP classification continues to be under discussion, and experts' panels strive for consensus. Malignancy is also under discussion, and it is shown in this review that there is a long-standing historical reason for that. Surgery is the main tool in the treatment armamentarium for PMP, and the only therapy with potential curative option.
PubMed: 31572561
DOI: 10.4240/wjgs.v11.i9.358 -
Frontiers in Oncology 2021To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), CA19-9, CA724, and CA242 in serum and ascites of pseudomyxoma peritonei...
PURPOSE
To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), CA19-9, CA724, and CA242 in serum and ascites of pseudomyxoma peritonei (PMP) patients and evaluate the predictive value of these elevated biomarkers in pathological grade, completeness of cytoreduction (CC), and survival.
METHODS
From May 2009 to October 2019, a total of 512 patients diagnosed with PMP through pathology in Aerospace Center Hospital were enrolled. The serum and ascites tumor biomarkers were obtained. The diagnostic values between serum and ascites biomarkers in pathology and CC were compared by the receiver operating characteristic (ROC) curves. The correlation between pathology, cytoreduction, and biomarkers was calculated by univariate and multivariate logistic regression. The associations between different numbers of elevated biomarkers and survival status were examined using univariate and multivariate backward Cox proportional hazard regression models.
RESULTS
The results showed that the areas under the ROC curves (AUROC) in the diagnosis of CC were 0.798 (95% CI: 0.760-0.836) and 0.632 (95% CI: 0.588-0.676) in serum and ascites biomarkers, respectively. The elevated serum and ascites biomarkers were independent risk factors for both pathology and CC. The 1-year, 3-year, and 5-year survival rates were 89.07%, 73.22%, and 66.94%, respectively. Longer survival was observed in patients who had less than two elevated serum biomarkers compared with those with 2-3 and 4-5 elevated serum biomarkers (p < 0.001).
CONCLUSION
CEA, CA125, CA19-9, CA724, and CA242 in serum and ascites can be used to judge the severity and predict the resectability. Furthermore, different numbers of elevated biomarkers can help determine the prognosis of PMP.
PubMed: 34733775
DOI: 10.3389/fonc.2021.594763 -
Experimental Hematology & Oncology Dec 2023Pseudomyxoma peritonei (PMP) is a rare disease characterized by a massive accumulation of mucus in the peritoneal cavity. The only effective treatment is aggressive...
Pseudomyxoma peritonei (PMP) is a rare disease characterized by a massive accumulation of mucus in the peritoneal cavity. The only effective treatment is aggressive surgery, aimed at removing all visible tumors. However, a high percentage of patients relapse, with subsequent progression and death. Recently, there has been an increase in therapies that target mutated oncogenic proteins. In this sense, KRAS has been reported to be highly mutated in PMP, with KRAS being the most common subtype. Here, we tested the efficacy of a small-molecule KRAS inhibitor, MRTX1133, in a high-grade PMP xenograft mouse model carrying a KRAS mutation. The results obtained in this work showed a profound inhibition of tumor growth, which was associated with a reduction in cell proliferation, an increase in apoptosis, and a reduction in the MAPK and PI3K/AKT/mTOR signaling pathways. In conclusion, these results demonstrate the high potency and efficacy of MRTX1133 in KRAS-PMP tumors and provide a rationale for clinical trials.
PubMed: 38066554
DOI: 10.1186/s40164-023-00465-4 -
Pseudomyxoma peritonei leading to "jelly belly" abdomen: a case report and review of the literature.Journal of Medical Case Reports Jun 2024Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management.
CASE PRESENTATION
A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases.
CONCLUSIONS
Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management.
Topics: Humans; Female; Pseudomyxoma Peritonei; Adult; Peritoneal Neoplasms; Tomography, X-Ray Computed; Cytoreduction Surgical Procedures; Ovarian Neoplasms; Ascites; Hysterectomy; Treatment Outcome
PubMed: 38937808
DOI: 10.1186/s13256-024-04612-1 -
Frontiers in Oncology 2022Pseudomyxoma peritonei is a rare disease that presents as a malignant tumor on the peritoneal surface. Cytoreductive surgery combined with hyperthermic intraperitoneal...
BACKGROUND
Pseudomyxoma peritonei is a rare disease that presents as a malignant tumor on the peritoneal surface. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the standard treatment for this disease and frequently requires a red blood cell transfusion. However, due to the limited collection and supply of allogeneic blood, surgical treatment may be delayed due to inadequate preparation of allogeneic blood in the course of clinical treatment. This study aimed to evaluate the safety and efficacy of transfusion of stored autologous blood in patients with low-grade pseudomyxoma peritonei.
METHODS
Pseudomyxoma peritonei patients who received cytoreductive surgery combined with heat-infused peritoneal chemotherapy were divided into two groups: transfusion of allogeneic blood and transfusion of stored autologous blood. A comparison of the differences in multiple factors between the two groups was performed, including tumor recurrence, survival time, hemoglobin and hematocrit levels, coagulation function (prothrombin time, activated partial thromboplastin time, and fibrinogen), total hospital stay duration, and incidence of serious adverse events after surgery.
RESULTS
Propensity scores matching analysis yielded 34 patients with allogeneic blood transfusion and 34 patients with stored autologous blood transfusion. Comparison analysis did not show statistical differences in several factors, including age, tumor grade, tumor recurrence rate after surgery, etc., between the two groups. The cytoreductive degree was considered an independent risk factor for tumor recurrence. The pseudomyxoma peritonei patients in the autologous transfusion group had a higher 5-year survival rate and a longer survival time. Moreover, transfusion of stored autologous blood did not increase the rate of tumor recurrence, or the total hospital stay duration after surgery, the hemoglobin level and coagulation function were well stabilized within 24 h after surgery, and there was a low incidence of serious adverse events.
CONCLUSION
The clinical application of transfusion of stored autologous blood in pseudomyxoma peritonei patients is safe and effective.
PubMed: 36276137
DOI: 10.3389/fonc.2022.1022426 -
Indian Journal of Surgical Oncology Jun 2016The combined treatment concept of cytoreductive surgery (CRS) and Hyperthermic intraperitoneal chemotherapy (HIPEC) has shown to be an efficient therapeutic option for... (Review)
Review
The combined treatment concept of cytoreductive surgery (CRS) and Hyperthermic intraperitoneal chemotherapy (HIPEC) has shown to be an efficient therapeutic option for selected patients with primary and secondary peritoneal carcinomatosis (PC). This strategy represents the standard of care for diseases like pseudomyxoma peritonei and peritoneal mesothelioma, and offers the best long-term results for PC from colorectal cancer. Despite these results, skepticism exists regarding this therapeutic approach partly because of its perceived high toxicity. In this article, we review the current evidence on complications that can occur after CRS and HIPEC and the risk factors associated with increased incidence of morbidity and mortality.
PubMed: 27065713
DOI: 10.1007/s13193-016-0504-6 -
Diagnostic Pathology Dec 2021As a rare clinical tumor syndrome with an indolent clinical course and lack of pathognomonic symptoms, pseudomyxoma peritonei (PMP) is usually diagnosed at an advanced...
BACKGROUND
As a rare clinical tumor syndrome with an indolent clinical course and lack of pathognomonic symptoms, pseudomyxoma peritonei (PMP) is usually diagnosed at an advanced stage. In-depth pathological analysis is essential to assess tumor biological behaviors, assist treatment decision, and predict the clinical prognosis of PMP. The tumor-stroma ratio (TSR) is a promising prognostic parameter based on the tumor and stroma. This study explored the relationship between TSR and the pathological characteristics and prognosis of PMP.
METHODS
PMP patients with complete data who underwent cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy were enrolled. The TSR of postoperative pathological images was quantitatively analyzed by Image-Pro Plus. Then the relationship between TSR and the clinicopathological characteristics, immunohistochemical characteristics and prognosis of PMP was analyzed.
RESULTS
Among the 50 PMP patients included, there were 27 males (54.0%) and 23 females (46.0%), with a median age of 55 (range: 31-76) years. 25 (50.0%) patients were diagnosed with low-grade PMP (LG-PMP), and 25 (50.0%) were diagnosed with high-grade PMP (HG-PMP). There were 4 (8.0%) patients with vascular tumor emboli, 3 (6.0%) patients with nerve invasion, and 5 (10.0%) patients with lymph node metastasis. The immunohistochemical results showed that the Ki67 label index was < 25% in 18 cases (36.0%), 25 - 50% in 18 cases (36.0%) and > 50% in 14 cases (28.0%). The range of TSR was 2 - 24% (median: 8%). The cutoff value of TSR was 10% based on the receiver operating characteristic (ROC) curve and X-Tile analysis. There were 31 (62.0%) cases with TSR < 10% and 19 (38.0%) cases with TSR ≥ 10%. The TSR was closely related to histopathological type (P < 0.001) and Ki67 label index (P < 0.001). Univariate analysis showed that preoperative carcinoembryonic antigen (CEA), preoperative carbohydrate antigen 19-9, pathological type, vascular tumor emboli and TSR influenced the prognosis of PMP patients (P < 0.05). Multivariate analysis showed that preoperative CEA, vascular tumor emboli and the TSR were independent prognostic factors.
CONCLUSIONS
The TSR could be a new independent prognosticator for PMP.
Topics: Adult; Aged; Biomarkers, Tumor; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermic Intraperitoneal Chemotherapy; Immunohistochemistry; Male; Middle Aged; Predictive Value of Tests; Pseudomyxoma Peritonei; Stromal Cells; Time Factors; Treatment Outcome
PubMed: 34895284
DOI: 10.1186/s13000-021-01177-1 -
World Journal of Gastroenterology Jun 2012To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei (PMP) in Chinese patients.
AIM
To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei (PMP) in Chinese patients.
METHODS
The clinicopathologic features and follow-up data of 92 patients with PMP were reviewed and retrospectively analyzed. The cases were categorized into three groups: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D). The log-rank test was used to analyze survival for each group and various clinicopathological parameters. Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.
RESULTS
The median age at diagnosis was 51.9 years (range: 22-76 years). The median follow up was 124 mo. The 3-, 5- and 10-year survival rates were 74.0%, 67.4% and 49.1%, respectively. There were 49 (53.2%) patients with DPAM, 26 (28.3%) with PMCA-I and 17 (18.5%) with PMCA. Patients with DPAM, PMCA-I/D and PMCA exhibited statistically significant difference in survival (P = 0.001). The 3 year survival for DPAM, PMCAI/D and PMCA was 97.0%, 80.0% and 67.0%, respectively; the 5 year survival was 80.0%, 67.0% and 50.0%, respectively; and the 10 year survival was 65.0%, 28.0% and 14.0%, respectively. Survival rate was significantly lowest in patients < 40 age years of age (P = 0.011). Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival. Patients with appendiceal mucinous adenocarcinoma (MACA) showed the significantly poorer prognosis (P = 0.011). Multivariate analysis showed that pathological classification, age, appendiceal tumor were significant related to overall survival.
CONCLUSION
The clinical process "PMP" should be pathologically classified into DPAM, PMCA and PMCA-I/D. Pathological classification, age, appendiceal MACA are survival independent predictors in Chinese patients with PMP.
Topics: Adult; Age Factors; Aged; Appendiceal Neoplasms; Asian People; China; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Multivariate Analysis; Peritoneal Neoplasms; Prognosis; Proportional Hazards Models; Pseudomyxoma Peritonei; Retrospective Studies; Risk Assessment; Risk Factors; Terminology as Topic; Time Factors; Young Adult
PubMed: 22791943
DOI: 10.3748/wjg.v18.i24.3081 -
Pleura and Peritoneum Jun 2023The Peritoneal Surface Oncology Group International (PSOGI) consensus subdivided pseudomyxoma peritonei (PMP) into four groups according to histopathological features....
Correlation between PSOGI pathological classification and survival outcomes of patients with pseudomyxoma peritonei treated using cytoreductive surgery and HIPEC: national referral centre experience and literature review.
OBJECTIVES
The Peritoneal Surface Oncology Group International (PSOGI) consensus subdivided pseudomyxoma peritonei (PMP) into four groups according to histopathological features. The aim of this paper is to report survival outcomes after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) from a national referral centre and to correlate the PSOGI classification with survival.
METHODS
A retrospective study of a prospectively maintained database was performed. Consecutive patients treated with CRS + HIPEC for PMP of appendiceal origin were included (September-2013 to December-2021). Pathological features of the peritoneal disease were used to classify patients into the four groups proposed by PSOGI. Survival analysis was performed to evaluate the correlation of pathology on overall survival (OS) and disease-free survival (DFS).
RESULTS
Overall, 104 patients were identified; 29.6 % were reclassified as acellular mucin (AM), 43.9 % as low-grade mucinous carcinoma peritonei (LGMCP), 22.4 % as high-grade MCP (HGMCP) and 4.1 % as HGMCP with signet ring cells (HGMCP-SRC). Median PCI and rate of optimal cytoreduction were 19 and 82.7 %, respectively. Median OS and DFS were not reached, 5-year OS and DFS were 88.6(SD 0.04) % and 61.6(SD 0.06) %, respectively. Log-Rank test revealed significant differences in terms of OS and DFS across the different histological subgroups (p<0.001 in both cases). However, histology did not retain its significance in the multivariate analysis for OS or DFS (p=0.932 and p=0.872, respectively).
CONCLUSIONS
Survival outcomes after CRS + HIPEC for PMP are excellent. The PSOGI pathological classification correlates with OS and DFS, but differences were not significant at multivariate analysis when adjusted for other prognostic factors.
PubMed: 37304162
DOI: 10.1515/pp-2023-0001