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The Lancet. Haematology Apr 2016Multicentric Castleman's disease describes a group of poorly understood lymphoproliferative disorders driven by proinflammatory hypercytokinaemia. Patients have... (Review)
Review
BACKGROUND
Multicentric Castleman's disease describes a group of poorly understood lymphoproliferative disorders driven by proinflammatory hypercytokinaemia. Patients have heterogeneous clinical features, characteristic lymph node histopathology, and often deadly multiple organ dysfunction. Human herpesvirus 8 (HHV8) causes multicentric Castleman's disease in immunosuppressed patients. The cause of HHV8-negative multicentric Castleman's disease is idiopathic; such cases are called idiopathic multicentric Castleman's disease. An absence of centralised information about idiopathic multicentric Castleman's disease represents a major challenge for clinicians and researchers. We aimed to characterise clinical features of, treatments for, and outcomes of idiopathic multicentric Castleman's disease.
METHODS
We did a systematic literature review and searched PubMed, the Cochrane database, and ClinicalTrials.gov from January, 1995, with keywords including "Castleman's disease" and "giant lymph node hyperplasia". Inclusion criteria were pathology-confirmed Castleman's disease in multiple nodes and minimum clinical and treatment information on individual patients. Patients with HHV8 or HIV infection or diseases known to cause Castleman-like histopathology were excluded.
FINDINGS
Our search identified 626 (33%) patients with HHV8-negative multicentric Castleman's disease from 1923 cases of multicentric Castleman's disease. 128 patients with idiopathic multicentric Castleman's disease met all inclusion criteria for the systematic review. Furthermore, aggregated data for 127 patients with idiopathic multicentric Castleman's disease were presented from clinical trials, which were excluded from primary analyses because patient-level data were not available. Clinical features of idiopathic multicentric Castleman's disease included multicentric lymphadenopathy (128/128), anaemia (79/91), elevated C-reactive protein (65/79), hypergammaglobulinaemia (63/82), hypoalbuminaemia (57/63), elevated interleukin 6 (57/63), hepatomegaly or splenomegaly (52/67), fever (33/64), oedema, ascites, anasarca, or a combination (29/37), elevated soluble interleukin 2 receptor (20/21), and elevated VEGF (16/20). First-line treatments for idiopathic multicentric Castleman's disease included corticosteroids (47/128 [37%]), cytotoxic chemotherapy (47/128 [37%]), and anti-interleukin 6 therapy (11/128 [9%]). 49 (42%) of 116 patients failed first-line therapy, 2-year survival was 88% (95% CI 81-95; 114 total patients, 12 events, 36 censored), and 27 (22%) of 121 patients died by the end of their observed follow-up (median 29 months [IQR 12-50]). 24 (19%) of 128 patients with idiopathic multicentric Castleman's disease had a diagnosis of a separate malignant disease, significantly higher than the frequency expected in age-matched controls (6%).
INTERPRETATION
Our systematic review provides comprehensive information about clinical features, treatment, and outcomes of idiopathic multicentric Castleman's disease, which accounts for at least 33% of all cases of multicentric Castleman's disease. Our findings will assist with prompt recognition, diagnostic criteria development, and effective management of the disease.
FUNDING
None.
Topics: Castleman Disease; HIV Infections; Herpesviridae Infections; Herpesvirus 8, Human; Humans; Lymph Nodes
PubMed: 27063975
DOI: 10.1016/S2352-3026(16)00006-5 -
Hepatology (Baltimore, Md.) Apr 2023Immunotherapy-based regimes have changed the management of HCC. However, evidence of efficacy in patients with impaired liver function is unknown. This systematic review... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIMS
Immunotherapy-based regimes have changed the management of HCC. However, evidence of efficacy in patients with impaired liver function is unknown. This systematic review and meta-analysis assesses survival of HCC patients and liver dysfunction treated with immunotherapy-based regimens.
METHODS
Systematic review and meta-analysis of original articles or abstracts reporting survival of HCC patients treated with immunotherapy according to liver function between 2017 and 2022. Overal survival (OS) according to restricted mean survival time (RMST) and median OS, and hazard ratio (HR) of Child-Pugh B or B/C versus Child-Pugh A were assessed while considering the line of treatment.
RESULTS
Of the 2218 articles considered, 15 articles recruiting 2311 patients were included. Of these, 639 (27.7%) were Child-Pugh B and 34 (1.5%) C. RMST was 8.36 (95% CI, 6.15-10.57; I2 =93%) months, estimated from 8 studies. The HR was reported in 8 studies for survival between Child-Pugh B versus Child-Pugh A and metanalysis disclosed a 1.65 HR (95% CI,1.45-1.84; I2 =0% heterogeneity; p = 0.45). Treatment line data were available for 47% of the patients and 3 studies included patients treated with atezolizumab-bevacizumab in the first line.
CONCLUSIONS
The high heterogeneity across studies reflects the incapacity of the current evidence to support the indication of immunotherapy in HCC patients with relevant liver dysfunction. It is mandatory to report complementary information to Child-Pugh classification such as prior liver decompensation, use of concomitant medication to control ascites, or signs of clinically significant portal hypertension to allow better patient stratification in future studies.
Topics: Humans; Carcinoma, Hepatocellular; Liver Neoplasms; Immunotherapy
PubMed: 36632997
DOI: 10.1097/HEP.0000000000000030 -
Acta Gastro-enterologica Belgica Dec 2014There is a common misconception that malignant ascites is equivalent to peritoneal carcinomatosis. It seems that malignancy-related ascites is a more appropriate... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND STUDY AIMS
There is a common misconception that malignant ascites is equivalent to peritoneal carcinomatosis. It seems that malignancy-related ascites is a more appropriate description of malignant ascites, which is difficult to confirm. Carcinoembryonic antigen, a glycoprotein tumor marker shed by malignant cells, increases in a wide range of gastrointestinal malignancies. We carried out the current meta-analysis to determine carcinoembryonic antigen accuracy in the diagnosis of malignancy-related ascites.
PATIENTS AND METHODS
Pubmed/Medline and SCOPUS were searched using these search terms: malignan* AND ascites AND (CEA OR carcinoembryonic). The outcome of interest was carcino-embryonic antigen accuracy in the differentiation of malignancy-related ascites and nonmalignant ascites.
RESULTS
Seven studies were included in this systematic review. Pooled diagnostic indices using random-effects model were as follows: sensitivity 43.1% [381-48.3]; specificity 95.5% [93-97.3]; LR+ (positive likelihood ratio) 7.33 [4.58-11.73]; LR- (negative likelihood ratio) 0.6 [0.54-0.68]; and DOR (diagnostic odds ratio) 12.93 [7.58-22].
CONCLUSIONS
Carcinoembryonic antigen of the ascitic fluid does not seem to be sensitive enough to diagnose malignancy-related ascites. However, due to high specificity, the positive predictive value of this marker is high and the higher the level of carcino-embryonic antigen, the more likely it is to be malignancy-related. Nevertheless, a negative test result cannot definitely rule out the malignancy.
Topics: Ascites; Ascitic Fluid; Carcinoembryonic Antigen; Humans; Liver Neoplasms; Peritoneal Neoplasms
PubMed: 25682632
DOI: No ID Found -
United European Gastroenterology Journal Oct 2023Transjugular intrahepatic portosystemic shunts (TIPS) in patients with hepatocellular carcinoma (HCC) may improve access to curative therapies, treat portal hypertension...
BACKGROUND/AIMS
Transjugular intrahepatic portosystemic shunts (TIPS) in patients with hepatocellular carcinoma (HCC) may improve access to curative therapies, treat portal hypertension (PH)-related complications without worsening liver function, and increase overall survival. Data on the efficacy and safety of TIPS to treat PH complications in HCC patients, as well as the HCC treatment response, were evaluated.
METHODS
Studies reporting efficacy in controlling bleeding/ascites or response to HCC therapy, safety, and survival in patients with HCC and TIPS were searched systematically on PubMed and Embase. An extraction of articles using predefined data fields and quality indicators was used.
RESULTS
We selected 19 studies and found 937 patients treated for ascites/bleeding and 177 evaluating HCC treatment response. Over half were under 5 cm and solitary lesions, and most studies included tumours with portal vein thrombosis. Regarding PH studies, TIPS resolved bleeding/ascites in >60% of patients, more effective for bleeding. There were no lethal complications reported and procedural bleeding occurred in <5%. Hepatic encephalopathy occurred in 15%-30% within three months. In the HCC treatment-response studies, major complication rates were low with no mortality. In the studies that evaluated the response to transarterial chemoembolization, complete response rate of patients with TIPS varied from 16% to 75%. Liver transplantation rate varied from 8% to 80%, with >40% rate in half of the studies.
CONCLUSIONS
In the published studies, TIPS is effective in treating PH complications in patients with HCC. Prospective studies on TIPS placement in patients with HCC are urgently needed to evaluate the efficacy and safety of TIPS in this setting.
Topics: Humans; Carcinoma, Hepatocellular; Portasystemic Shunt, Transjugular Intrahepatic; Liver Neoplasms; Ascites; Prospective Studies; Esophageal and Gastric Varices; Treatment Outcome; Chemoembolization, Therapeutic; Hypertension, Portal
PubMed: 37736854
DOI: 10.1002/ueg2.12454 -
Gastroenterology Oct 2022Magnetic resonance elastography (MRE) is an accurate biomarker of liver fibrosis; however, limited data characterize its association with clinical outcomes. We conducted... (Meta-Analysis)
Meta-Analysis
Liver Stiffness on Magnetic Resonance Elastography and the MEFIB Index and Liver-Related Outcomes in Nonalcoholic Fatty Liver Disease: A Systematic Review and Meta-Analysis of Individual Participants.
BACKGROUND & AIMS
Magnetic resonance elastography (MRE) is an accurate biomarker of liver fibrosis; however, limited data characterize its association with clinical outcomes. We conducted an individual participant data pooled meta-analysis on patients with nonalcoholic fatty liver disease to evaluate the association between liver stiffness on MRE and liver-related outcomes.
METHODS
A systematic search identified 6 cohorts of adults with nonalcoholic fatty liver disease who underwent a baseline MRE and were followed for hepatic decompensation, hepatocellular carcinoma, and death. Cox and logistic regression were used to assess the association between liver stiffness on MRE and liver-related outcomes, including a composite primary outcome defined as varices needing treatment, ascites, and hepatic encephalopathy.
RESULTS
This individual participant data pooled meta-analysis included 2018 patients (53% women) with a mean (± standard deviation) age of 57.8 (±14) years and MRE at baseline of 4.15 (±2.19) kPa, respectively. Among 1707 patients with available longitudinal data with a median (interquartile range) of 3 (4.2) years of follow-up, the hazard ratio for the primary outcome for MRE of 5 to 8 kPa was 11.0 (95% confidence interval [CI]: 7.03-17.1, P < .001) and for ≥ 8 kPa was 15.9 (95% CI: 9.32-27.2, P < .001), compared with those with MRE <5 kPa. The MEFIB index (defined as positive when MRE ≥3.3 kPa and Fibrosis-4 ≥1.6) had a robust association with the primary outcome with a hazard ratio of 20.6 (95% CI: 10.4-40.8, P < .001) and a negative MEFIB had a high negative predictive value for the primary outcome, 99.1% at 5 years. The 3-year risk of incident hepatocellular carcinoma was 0.35% for MRE <5 kPa, 5.25% for 5 to 8 kPa, and 5.66% for MRE ≥8 kPa, respectively.
CONCLUSION
Liver stiffness assessed by MRE is associated with liver-related events, and the combination of MRE and Fibrosis-4 has excellent negative predictive value for hepatic decompensation. These data have important implications for clinical practice.
Topics: Adult; Aged; Biomarkers; Carcinoma, Hepatocellular; Elasticity Imaging Techniques; Female; Fibrosis; Humans; Liver; Liver Cirrhosis; Liver Neoplasms; Magnetic Resonance Imaging; Male; Middle Aged; Non-alcoholic Fatty Liver Disease
PubMed: 35788349
DOI: 10.1053/j.gastro.2022.06.073 -
Abdominal Radiology (New York) May 2022Imaging of the peritoneum and related pathology is a challenge. Among peritoneal diseases, malignant peritoneal mesothelioma (MPeM) is an uncommon tumor with poor... (Review)
Review
PURPOSE
Imaging of the peritoneum and related pathology is a challenge. Among peritoneal diseases, malignant peritoneal mesothelioma (MPeM) is an uncommon tumor with poor prognosis. To date, there are no specific guidelines or imaging protocols dedicated for the peritoneum and MPeM. The objective of this study was to analyze the literature describing imaging modalities used for MPeM to determine their relative clinical efficacy and review commonly reported imaging features of MPeM to promote standardized reporting.
METHODS
We performed a systematic review of original research articles discussing imaging modalities in MPeM from 1999 to 2020. Effectiveness measures and common findings were compared across imaging modalities.
RESULTS
Among 582 studies analyzed, the most-used imaging modality was CT (54.3%). In the differentiation of MPeM from peritoneal carcinomatosis, one study found CT had a diagnostic sensitivity of 53%, specificity of 100%, and accuracy of 68%. Two studies found fluorodeoxyglucose positron emission tomography (FDG-PET) had sensitivity of 86-92%, specificity of 83-89%, and accuracy of 87-89%. Another study found magnetic resonance imaging (MRI) was the best predictor of the peritoneal carcinomatosis index. Characteristics shown to best differentiate MPeM from other diseases included ascites, peritoneal thickening, mesenteric thickening, pleural plaques, maximum tumor dimension, and number of masses.
CONCLUSION
Most published MPeM imaging studies utilized CT. PET/CT or MRI appear promising, and future studies should compare effectiveness of these modalities. MPeM imaging reports should highlight ascites, number of and maximum tumor dimension, peritoneal/mesenteric thickening, and associated pleural plaques, allowing for better aggregation of MPeM imaging data across studies.
Topics: Ascites; Humans; Mesothelioma; Peritoneal Neoplasms; Positron Emission Tomography Computed Tomography; Tomography, X-Ray Computed
PubMed: 35257201
DOI: 10.1007/s00261-022-03464-x -
World Journal of Gastrointestinal... Jun 2021Chylous ascites is a rare complication in colorectal surgery with limited evidence.
BACKGROUND
Chylous ascites is a rare complication in colorectal surgery with limited evidence.
AIM
To systematically review all available evidence to describe the incidence, clinical presentation, risk factors and management strategies.
METHODS
The systematic review was performed through PubMed, MEDLINE, EMBASE and Cochrane and cross-checked up to November 2020. The data collated included: Demographics, indications (benign malignant), site of disease, surgical approach, extent of lymphadenectomy, day to and method of diagnosis of chylous ascites and management strategies.
RESULTS
A total of 28 studies were included in the final analysis (426 cases). Patient age ranged from 31 to 89 years. All except one case were performed for malignancy. Of the 426 cases, 195 were right-colonic, 121 left-colonic, 103 pelvic surgeries and 7 others. The majority were diagnosed during the same inpatient stay by recognition of typical drain appearance and increased volume. Three cases were diagnosed during outpatient visits with increased abdominal distention and subsequently underwent paracentesis. Most cases were managed successfully non-operatively (fasting with prolonged drainage, total parenteral nutrition, somatostatin analogues or a combination of these). Only three cases required surgical intervention after failing conservative management and subsequently resolved completely. Risk factors identified include: Right-colonic surgery/ tumour location, extent of lymphadenectomy and number of lymph nodes harvested.
CONCLUSION
Chylous ascites after colorectal surgery is a relatively rare complication. Whilst the majority of cases resolved without surgical intervention, preventative measures should be undertaken such as meticulous dissection and clipping of lymphatics during lymphadenectomy to prevent morbidity.
PubMed: 34194616
DOI: 10.4240/wjgs.v13.i6.585 -
Cancers Mar 2024Malignant Brenner tumors are rare ovarian tumors, accounting for less than 1% of malignant ovarian neoplasms. The aim of this manuscript is to systematically review the... (Review)
Review
BACKGROUND
Malignant Brenner tumors are rare ovarian tumors, accounting for less than 1% of malignant ovarian neoplasms. The aim of this manuscript is to systematically review the current literature concerning malignant Brenner tumors.
METHODS
We searched three medical databases (PubMed, Scopus, and Web of Science) for relevant articles published until 15 September 2023.
RESULTS
After applying inclusion and exclusion criteria, 48 manuscripts describing 115 cases were included in this study from the English literature.
CONCLUSIONS
We analyzed the demographic, clinical, pathological, and oncological characteristics of 115 patients with malignant Brenner tumors. The statistical analysis showed that recurrence was marginally statistically significantly related to tumor stage and was more common in patients with ascites and in women with abnormal CA-125 levels; patients that were treated with lymphadenectomy had better disease-specific survival.
PubMed: 38539441
DOI: 10.3390/cancers16061106 -
European Journal of Surgical Oncology :... Mar 2022Many prognostic models for Hepatocellular Carcinoma (HCC) have been developed to inform patients and doctors about individual prognosis. Previous reviews of these models... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Many prognostic models for Hepatocellular Carcinoma (HCC) have been developed to inform patients and doctors about individual prognosis. Previous reviews of these models were qualitative and did not assess performance at external validation. We assessed the performance of prognostic models for HCC and set a benchmark for biomarker studies.
METHODS
All externally validated models predicting survival for patients with resected HCC were systematically reviewed. After selection, we extracted descriptive statistics and aggregated c-indices using meta-analysis.
RESULTS
Thirty-eight validated prognostic models were included. Models used on average 7 (IQR:4-9) prognostic factors. Tumor size, tumor number, and vascular invasion were almost always included. Alpha-fetoprotein (AFP) was commonly incorporated since 2007. Recently, the more subjective items ascites and encephalopathy have been dropped. Eight established models performed poor to moderate at external validation, with a pooled C-index below 0.7; including the Barcelona Clinic Liver Cancer (BCLC) system, the American Joint Committee on Cancer (AJCC) 7th edition, the Cancer of the Liver Italian (CLIP) Program, and the Japan Integrated Staging (JIS) score. Out of 24 prognostic models predicting OS, only 6 (25%) had good performance at external validation with pooled C-indices above 0.7; the Li-post (0.77), Li-OS (0.74), Yang-pre (0.74), Yang-post (0.76), Shanghai-score (0.70), and Wang-nomogram (0.71). Models improved over time, but overall performance and study quality remained low.
CONCLUSIONS
Six validated prognostic models demonstrated good performance for predicting survival after resection of HCC. These models can guide patients and doctors and are a benchmark for future models incorporating novel biomarkers.
Topics: Biomarkers; Carcinoma, Hepatocellular; China; Humans; Liver Neoplasms; Neoplasm Staging; Prognosis
PubMed: 34602315
DOI: 10.1016/j.ejso.2021.09.012 -
The Cochrane Database of Systematic... Aug 2017Cystic fibrosis is an autosomal recessive inherited defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in abnormal regulation of... (Review)
Review
BACKGROUND
Cystic fibrosis is an autosomal recessive inherited defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in abnormal regulation of salt and water movement across the membranes. In the liver this leads to focal biliary fibrosis resulting in progressive portal hypertension and end-stage liver disease in some individuals. This can be asymptomatic, but may lead to splenomegaly and hypersplenism, development of varices and variceal bleeding, and ascites; it has negative impact on overall nutritional status and respiratory function in this population. Prognosis is poor once significant portal hypertension is established. The role and outcome of various interventions for managing advanced liver disease (non-malignant end stage disease) in people with cystic fibrosis is currently unidentified.
OBJECTIVES
To review and assess the efficacy of currently available treatment options for preventing and managing advanced liver disease in children and adults with cystic fibrosis.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books.Date of last search: 06 April 2017.We also searched the reference lists of relevant articles and reviews and online trials registries. Date of last search: 04 January 2017.
SELECTION CRITERIA
Any published and unpublished randomised controlled trials and quasi-randomised controlled trials of advanced liver disease in cystic fibrosis with cirrhosis or liver failure, portal hypertension or variceal bleeding (or both).
DATA COLLECTION AND ANALYSIS
Authors independently examined titles and abstracts to identify potentially relevant trials, but none were eligible for inclusion in this review.
MAIN RESULTS
A comprehensive search of the literature did not identify any published eligible randomised controlled trials.
AUTHORS' CONCLUSIONS
In order to develop the best source of evidence, there is a need to undertake randomised controlled trials of interventions for preventing and managing advanced liver disease in adults and children with cystic fibrosis.
Topics: Cystic Fibrosis; Humans; Liver Diseases
PubMed: 28850173
DOI: 10.1002/14651858.CD012056.pub2