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Journal of Visceral Surgery Feb 2019Pseudomyxoma peritonei is a rare tumor characterized by the presence of mucous disseminated throughout the peritoneal cavity generally arising from the rupture of an...
Pseudomyxoma peritonei is a rare tumor characterized by the presence of mucous disseminated throughout the peritoneal cavity generally arising from the rupture of an appendicular mucocele. Liver scalloping is a highly suggestive image of pseudomyxoma and corresponds to the indentation of the liver compressed by the gelatinous mucin.
Topics: Constriction, Pathologic; Humans; Liver; Pseudomyxoma Peritonei
PubMed: 30522825
DOI: 10.1016/j.jviscsurg.2018.09.001 -
Chirurgia (Bucharest, Romania : 1990) 2015Appendicular mucocele, a cystic dilatation of the appendix, is a rare disease, but unfortunately about 1/10 of cases evolves into pseudomyxoma peritonei. (Review)
Review
INTRODUCTION
Appendicular mucocele, a cystic dilatation of the appendix, is a rare disease, but unfortunately about 1/10 of cases evolves into pseudomyxoma peritonei.
METHODS
We performed a prospective study between 1 January 2010 to 31 December 2014 in order to track the incidence, symptoms, and circumstances of diagnosis, treatment and evolution of these rare tumors.
RESULTS
A total of seven patients underwent curative surgery for a mucocele of the appendix: one woman and six men with an average age of 59.71 years. Clinical signs, present in two cases, were uncharacteristic. Ultrasound performed in all cases, could guide diagnosis in 5 cases. CT performed in 5 cases diagnosed only two cases. All cases were operated on: the open approach was used in four cases and a minimally invasive in three cases. We performed two right colectomies, an open appendectomy associated to anterior resection of the rectum, two laparoscopic appendectomies and two appendectomies and cecum resection with stapler, one by open approach and one by a minimally invasive approach. Intraoperative spillage of mucinous tumor did not occur in any case. The mean hospital stay was 5.7 days. Postoperative complications were present in 1 case (14.2%): wound infection. The average follow-up period was 40.28 months. (Range 6 to 48 months). No tumor recurrence or readmission, such as pseudomyxoma peritonei, has occurred.
CONCLUSIONS
Appendicular mucocele is a rare entity; it can be found incidentally and it can mimic acute appendicitis, appendicular plastron or cecum tumor. Once diagnosed, surgical treatment is required for fear of perforation, tumor evolution and the emergence of the rule of complications. Laparoscopic approach in selected cases can be used, accompanied by safety measures to avoid iatrogenic perforation and peritoneal and parietal seeding.
Topics: Adult; Aged; Appendectomy; Appendix; Colectomy; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Incidence; Laparoscopy; Length of Stay; Male; Middle Aged; Mucocele; Prospective Studies; Risk Factors; Romania; Treatment Outcome; Wound Infection
PubMed: 26713827
DOI: No ID Found -
Journal of Visualized Experiments : JoVE Dec 2022Pseudomyxoma peritonei (PMP) is a rare condition that results from the dissemination of a mucinous primary tumor and the resultant accumulation of mucin-secreting tumor...
Pseudomyxoma peritonei (PMP) is a rare condition that results from the dissemination of a mucinous primary tumor and the resultant accumulation of mucin-secreting tumor cells in the peritoneal cavity. PMP can arise from various types of cancers, including appendiceal, ovarian, and colorectal, though appendiceal neoplasms are by far the most common etiology. PMP is challenging to study due to its (1) rarity, (2) limited murine models, and (3) mucinous, acellular histology. The method presented here allows real-time visualization and interrogation of these tumor types using patient-derived ex vivo organotypic slices in a preparation where the tumor microenvironment (TME) remains intact. In this protocol, we first describe the preparation of tumor slices using a vibratome and subsequent long-term culture. Second, we describe confocal imaging of tumor slices and how to monitor functional readouts of viability, calcium imaging, and local proliferation. In short, slices are loaded with imaging dyes and are placed in an imaging chamber that can be mounted onto a confocal microscope. Time-lapse videos and confocal images are used to assess the initial viability and cellular functionality. This procedure also explores translational cellular movement, and paracrine signaling interactions in the TME. Lastly, we describe a dissociation protocol for tumor slices to be used for flow cytometry analysis. Quantitative flow cytometry analysis can be used for bench-to-bedside therapeutic testing to determine changes occurring within the immune landscape and epithelial cell content.
Topics: Female; Humans; Animals; Mice; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Appendiceal Neoplasms; Ovary; Tumor Microenvironment
PubMed: 36571414
DOI: 10.3791/64620 -
Annals of Coloproctology Feb 2017Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been proposed for controlling peritoneal seeding metastasis in some kinds of...
PURPOSE
Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been proposed for controlling peritoneal seeding metastasis in some kinds of cancers, including those of colorectal origin, but their safety and oncological benefits are subjects of debate. We present our early experience with those procedures.
METHODS
Data were retrospectively collected from all patients with peritoneal carcinomatosis (PC) and pseudomyxoma peritonei (PMP) treated using CRS and HIPEC at Yonsei Cancer Center between July 2014 and July 2015. Short-term outcomes and risk factors for postoperative complications were analyzed.
RESULTS
Twenty-three patients with PC (n = 18) and PMP (n = 5) underwent CRS and HIPEC. Median follow-up and age were 2 months and 54 years, respectively. The median peritoneal carcinomatosis index score was 15, and CC0-1 was achieved in 78.3% of all patients. The median operation time and bleeding loss were 590 minutes and 570 mL, respectively. Grade-IIIa/grade-IIIb complications occurred in 4.3% (n = 1)/26.1% (n = 6) of the patients within 30 days postoperatively, and no 30-day mortalities were reported. Factors related to postoperative complications with CRS and HIPEC were number of organ resection (P = 0.013), longer operation time (P < 0.001), and amount of blood loss (P = 0.003). All patients treated with cetuximab for recurred colorectal cancer had grade-III postoperative complication.
CONCLUSION
Our initial experience with CRS and HIPEC presented about 30% grade-III postoperative complications. Therefore, expert surgeons need to perform those procedures with great caution in selected patients who might benefit from it.
PubMed: 28289659
DOI: 10.3393/ac.2017.33.1.16 -
World Journal of Surgical Oncology May 2024Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal...
BACKGROUND
Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). However, tubing blockages during HIPEC treatment pose a common challenge, impeding the smooth progression of therapy. Few studies to date have explored the incidence and risk factors of tube occlusion during HIPEC in patients with appendiceal PMP, as well as its adverse impact on postoperative complications.
METHODS
From October 2017 to June 2023, a total of 80 patients with appendiceal PMP undergoing combined CRS and HIPEC were included in this study. Tubing blockage events were strictly defined, with patients experiencing blockages during HIPEC treatment allocated to the study group, while those with unobstructed perfusion were assigned to the control group. A comparative analysis was conducted between the two groups regarding post-HIPEC health assessments and occurrence of complications. Risk factors for luminal occlusion during closed HIPEC procedures were identified through univariate and multivariate analysis of data from 303 HIPEC treatments.
RESULTS
Tubing blockages occurred in 41 patients (51.3%). The study group experienced prolonged gastrointestinal decompression time (4.1 ± 3.0 vs. 2.5 ± 1.7 days, P = 0.003) and prolonged time to bowel movement (6.1 ± 2.3 vs. 5.1 ± 1.8 days, P = 0.022) compared to the control group. There was no significant difference in the incidence of complications between the two groups. The 1-year survival rate postoperatively was 97%, and the 3-year survival rate was 81%, with no association found between tubing blockage and poorer survival. Additionally, In 303 instances of HIPEC treatment among these 80 patients, tube occlusion occurred in 89 cases (89/303, 29.4%). Multivariable logistic regression analysis revealed age, diabetes, hypertension, and pathology as independent risk factors for tube occlusion.
CONCLUSION
Tubing blockages are a common occurrence during HIPEC treatment, leading to prolonged postoperative gastrointestinal functional recovery time. When patients are elderly and have concomitant hypertension and diabetes, along with a histological type of low-grade mucinous tumor, the risk of tube occlusion increases. However, this study did not find a significant correlation between tubing blockage and the incidence of postoperative complications or overall patient survival.
Topics: Humans; Pseudomyxoma Peritonei; Female; Male; Middle Aged; Peritoneal Neoplasms; Appendiceal Neoplasms; Prognosis; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Follow-Up Studies; Postoperative Complications; Adult; Retrospective Studies; Combined Modality Therapy; Survival Rate; Aged; Risk Factors; Hyperthermia, Induced
PubMed: 38769546
DOI: 10.1186/s12957-024-03412-7 -
BMC Cancer Jan 2023To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP)...
BACKGROUND
To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP) patients relative to their diagnostic and predictive value.
METHODS
The study comprised 183 patients with pathologically confirmed appendiceal PMP, enrolled from May 2012 to June 2020, in Aerospace Center Hospital. Serum and ascites tumor markers were obtained, and their diagnostic values were compared by receiver operating characteristic (ROC) curves. The prognostic factors of appendiceal PMP with different pathologic subgroups were calculated by univariate and multivariate Cox proportional hazard regression models.
RESULTS
There were significant differences between the numbers of patients with positive CEA and CA199 in serum vs. ascites: p = 0.034 in CEA and p = 0.006 in CA199, respectively. The sensitivities with optimal cut-off values for ascites markers of CEA, CA199 and CA125 were 83.5%, 88.9% and 72.6%, respectively. CEA in ascites showed significant difference in the diagnosis of appendiceal PMP (p = 0.000); the areas under the ROC curves (AUROCs) and specificity were 0.725, 70.7%, respectively. Univariate analysis showed that the higher the ascites tumor markers, the poorer the survival (p = 0.014). Multivariate analysis indicated that completeness of cytoreduction (CCR), ascites CEA and pathological grade were independent risk factors for overall survival (OS).
CONCLUSION
CEA in ascites can be used to help specify the origin of PMP. Furthermore, elevation of ascites CEA, high pathological grade and incomplete cytoreduction predicted poor prognosis of appendiceal PMP.
Topics: Humans; Pseudomyxoma Peritonei; Prognosis; Carcinoembryonic Antigen; Biomarkers, Tumor; Peritoneal Neoplasms; Ascites; Appendiceal Neoplasms; CA-125 Antigen
PubMed: 36703100
DOI: 10.1186/s12885-023-10545-7 -
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 -
Cancers Jun 2021Pseudomyxoma peritonei (PMP) is a rare, slow-growing cancer characterized by progressive accumulation of intraperitoneal mucinous tumor deposits. Cytoreductive surgery...
Pseudomyxoma peritonei (PMP) is a rare, slow-growing cancer characterized by progressive accumulation of intraperitoneal mucinous tumor deposits. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) cures approximately 50% of patients, but in unresectable and recurrent cases, treatment options are limited. Anti-angiogenic treatment is being explored as a potential therapeutic option. Using PMP patient samples, microvessel densities (immunostaining for CD31 and CD105) and pro-angiogenic factors were analyzed, and the proliferative response upon incubation with human umbilical cord vascular endothelial cells (HUVEC) was determined. Growth inhibition by anti-angiogenic drugs was analyzed in patient-derived xenograft models of PMP. PMP tumor tissues were found to be highly vascularized and contained key pro-angiogenic factors, in particular related to vascular endothelial growth factor (VEGF) signaling, but interestingly, high levels of fibroblast growth factor 2 were also detected. HUVEC proliferation was stimulated upon incubation with fresh tumor samples and the observed proliferation could be inhibited by VEGF pathway inhibitor bevacizumab. In xenograft models the two VEGF pathway inhibitors, bevacizumab and aflibercept, inhibited tumor growth. This work reemphasizes the importance of angiogenesis as a major driver in PMP and strengthens the preclinical rationale for continued exploration of angiogenesis inhibition in the hope of providing novel treatment to a group of patients that have few other treatment options.
PubMed: 34198773
DOI: 10.3390/cancers13112819 -
Germs Jun 2021Gastrointestinal tuberculosis is an important form of extrapulmonary tuberculosis but liver involvement is uncommon. Hepatic tuberculosis can manifest in variable...
INTRODUCTION
Gastrointestinal tuberculosis is an important form of extrapulmonary tuberculosis but liver involvement is uncommon. Hepatic tuberculosis can manifest in variable manners including as a space occupying lesion, diffuse infiltration by granulomas (granulomatous hepatitis) and as liver abscess. Hepatic tuberculosis is usually associated with lesions elsewhere but the diagnosis is often not considered because of rarity.
CASE REPORT
We report two cases of hepatic involvement by tuberculosis: one in the form of liver abscess and the other having multiple hypodense hepatic lesions along with peritoneal involvement. The presence of ascites, visceral scalloping and multiple hypodense lesions resulted in a diagnostic confusion with disseminated malignancy but GeneXpert Ultra (Cepheid, USA) helped in making the diagnosis.
DISCUSSION
Hepatic tuberculosis should be considered in the differential diagnosis of hepatic space occupying lesions and abscesses. This is especially important in TB endemic regions and in patients where there is involvement of other organs especially the lungs.
CONCLUSIONS
Newer microbiological modalities including GeneXpert Ultra could help in making a diagnosis of hepatic tuberculosis.
PubMed: 34422701
DOI: 10.18683/germs.2021.1266 -
World Journal of Gastrointestinal... Jun 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard treatment. However, there are few studies and insufficient evidence regarding systemic chemotherapy of advanced PMP. Regimens for colorectal cancer are often used clinically, but there is no uniform standard for late-stage treatment.
AIM
To determine if bevacizumab combined with cyclophosphamide and oxaliplatin (Bev+CTX+OXA) is effective for treatment of advanced PMP. The primary study endpoint was progression-free survival (PFS).
METHODS
Retrospective analysis was conducted on the clinical data of patients with advanced PMP who received Bev+CTX+OXA regimen (bevacizumab 7.5 mg/kg ivgtt d1, oxaliplatin 130 mg/m ivgtt d1 and cyclophosphamide 500 mg/m ivgtt d1, q3w) in our center from December 2015 to December 2020. Objective response rate (ORR), disease control rate (DCR) and incidence of adverse events were evaluated. PFS was followed up. Kaplan-Meier method was used to draw survival curve, and log-rank test was used for comparison between groups. Multivariate Cox proportional hazards regression model was used to analyze the independent influencing factors of PFS.
RESULTS
A total of 32 patients were enrolled. After 2 cycles, the ORR and DCR were 3.1% and 93.7%, respectively. The median follow-up time was 7.5 mo. During the follow-up period, 14 patients (43.8%) had disease progression, and the median PFS was 8.9 mo. Stratified analysis showed that the PFS of patients with a preoperative increase in CA125 (8.9 2.1, = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 5.0, = 0.043) was significantly longer than that of the control group. Multivariate analysis showed that a preoperative increase in CA125 was an independent prognostic factor for PFS (HR = 0.245, 95%CI: 0.066-0.904, = 0.035).
CONCLUSION
Our retrospective assessment confirmed that the Bev+CTX+OXA regimen is effective in second- or posterior-line treatment of advanced PMP and that adverse reactions can be tolerated. A preoperative increase in CA125 is an independent prognostic factor of PFS.
PubMed: 37405093
DOI: 10.4240/wjgs.v15.i6.1149