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Familial Cancer Oct 2022Desmoid tumours (DT) are one of the main causes of death in patients with familial adenomatous polyposis (FAP). Surgical trauma is a risk factor for DT, yet a colectomy... (Meta-Analysis)
Meta-Analysis Review
Desmoid tumours (DT) are one of the main causes of death in patients with familial adenomatous polyposis (FAP). Surgical trauma is a risk factor for DT, yet a colectomy is inevitable in FAP to prevent colorectal cancer. This systematic review and meta-analysis aimed to synthesize the available evidence on DT risk related to type, approach and timing of colectomy. A search was performed in MEDLINE, EMBASE and the Cochrane Library. Studies were considered eligible when DT incidence was reported after different types, approaches and timing of colectomy. Twenty studies including 6452 FAP patients were selected, all observational. No significant difference in DT incidence was observed after IRA versus IPAA (OR 0.99, 95% CI 0.69-1.42) and after open versus laparoscopic colectomy (OR 0.88, 95% CI 0.42-1.86). Conflicting DT incidences were seen after early versus late colectomy and when analysing open versus laparoscopic colectomy according to colectomy type. Three studies reported a (non-significantly) higher DT incidence after laparoscopic IPAA compared to laparoscopic IRA, with OR varying between 1.77 and 4.09. A significantly higher DT incidence was observed in patients with a history of abdominal surgery (OR 3.40, 95% CI 1.64-7.03, p = 0.001). Current literature does not allow to state firmly whether type, approach, or timing of colectomy affects DT risk in FAP patients. Fewer DT were observed after laparoscopic IRA compared to laparoscopic IPAA, suggesting laparoscopic IRA as the preferred choice if appropriate considering rectal polyp burden. PROSPERO REGISTRATION NUMBER: CRD42020161424.
Topics: Humans; Fibromatosis, Aggressive; Colectomy; Adenomatous Polyposis Coli; Laparoscopy; Incidence; Proctocolectomy, Restorative
PubMed: 35022961
DOI: 10.1007/s10689-022-00288-y -
Digestive Surgery 2021The management of the pancreas in patients with duodenal trauma or duodenal tumors remains a controversial issue. Pancreas-preserving total duodenectomy (PPTD) requires...
BACKGROUND
The management of the pancreas in patients with duodenal trauma or duodenal tumors remains a controversial issue. Pancreas-preserving total duodenectomy (PPTD) requires a meticulous surgical technique. The most common indication is familial duodenal adenomatous polyposis (FAP). The aims of this study are to carry out a systematic review of the literature on the indications for PPTD and to highlight the risks and benefits compared with other more aggressive procedures.
SUMMARY
A systematic literature review was performed following PRISMA recommendations of studies published in PubMed, Embase, and Cochrane library until May 2019. Thirty articles describing 211 patients were chosen. The mean age was 48 years. The surgical indication in 75% of patients was FAP. The mean operating time was 329 min and mean intraoperative bleeding 412 mL. Postoperative morbidity rate was 49.7% (76% Clavien-Dindo
97.8%. Key Messages: PPTD is indicated for patients with benign and premalignant duodenal lesions without involvement of the pancreatic head. It is a feasible procedure offering an alternative to other more aggressive procedures in selected patients. Mortality is below 1.5%. Topics: Adenomatous Polyposis Coli; Digestive System Surgical Procedures; Duodenal Neoplasms; Duodenum; Humans; Pancreas; Postoperative Complications
PubMed: 34000717
DOI: 10.1159/000515718 -
Surgical Endoscopy Mar 2022This paper aimed to elucidate the etiologies of all primary ileostomy site malignancies published in the literature. (Review)
Review
BACKGROUND
This paper aimed to elucidate the etiologies of all primary ileostomy site malignancies published in the literature.
METHODS
A review of the literature was conducted following PRISMA guidelines by querying PubMed, Global Health, and Web of Science for articles published before November 2020. Search criteria contained broad terminology for ileostomy site neoplasms without language, date, or publication limitations. A full-text review of the abstracts confirmed primary malignant pathologies and was evaluated for study inclusion.
RESULTS
Literature search discovered 858 publications, with 76 meeting eligibility criteria. The final sample contained 91 patients, with equal males and females. The mean age of patients with ileostomy site malignancy was 62.0 ± 12.2, with an average ileostomy age of 29.4 ± 12.4. The most common indications for ileostomy creation were inflammatory bowel disease (IBD) (73.6%) and familial adenomatous polyposis (FAP) (20.9%). There was a total of eight ileostomy malignant pathologies reported, with adenocarcinoma being the most common (76.9%), followed by squamous cell carcinoma (SCC) (11.0%). Adenocarcinoma was diagnosed at a younger age than SCC (59.7 vs. 72.3) and developed over a shorter time (28.8 vs. 37.0). Patients with FAP almost exclusively developed adenocarcinoma (94.4%) at a younger stoma age (25.8 vs. 31.4) than those with IBD who developed seven diverse pathologies. With a median follow-up of 0.75 years, four patients developed disease recurrence and received oncologic resection of their cancer less often than the 55 negative patients (p = 0.04).
CONCLUSION
Ileostomy site malignancies are late-appearing complications that require curative surgery. Their presentation is associated with ileostomy duration and creation indication, such as FAP or IBD. We recommend screening at a stoma age ≥ 20 or patient age ≥ 50 for patients with FAP, while stoma age ≥ 25 or patient age ≥ 60 for IBD patients.
Topics: Adenocarcinoma; Adenomatous Polyposis Coli; Female; Humans; Ileostomy; Male; Neoplasm Recurrence, Local; Surgical Stomas
PubMed: 34997348
DOI: 10.1007/s00464-021-08847-7 -
Techniques in Coloproctology Sep 2022Ileal pouch-anal anastomosis (IPAA) is commonly used to restore gastrointestinal continuity after surgical treatment of mucosal ulcerative colitis (MUC) and familial... (Meta-Analysis)
Meta-Analysis Review
A systematic review and meta-analysis of the outcome of ileal pouch-anal anastomosis in patients with ulcerative colitis versus patients with familial adenomatous polyposis.
BACKGROUND
Ileal pouch-anal anastomosis (IPAA) is commonly used to restore gastrointestinal continuity after surgical treatment of mucosal ulcerative colitis (MUC) and familial adenomatous polyposis (FAP). The aim of the present systematic review was to compare the outcomes of patients with MUC and patients with FAP who underwent IPAA.
METHODS
A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-compliant systematic review was performed. PubMed, Scopus, and Web of Science were searched through December 2021. Cohort and randomized studies were eligible for inclusion if they directly compared patients with MUC and FAP who underwent IPAA. The main outcome measures were pouch failure, complications, and need for pouch excision or revision. ROBINS-I tool was used to assess the risk of bias across the studies. A random-effect meta-analysis was conducted.
RESULTS
Twenty-three studies (9200 patients) were included in this meta-analysis. Seven thousand nine hundred fifty (86.4%) had MUC and 1250 (13.6%) had FAP. The median age of patients was 33.1 years. The male to female ratio was 1.4:1. MUC had higher odds of pouchitis (OR 3.9, 95% CI 2.8-5.4, p < 0.001), stricture (OR 1.82, 95% CI 1.25-2.65, p = 0.002), fistula (OR 1.74, 95% CI 1.18-2.54, p = 0.004), and total complications (OR 1.89, 95% CI 1.3-2.77, p < 0.001) as compared to FAP. Both groups had similar odds of pelvic sepsis, leakage, pouch failure, excision, revision, and fecal incontinence.
CONCLUSIONS
Although patients with MUC undergoing IPAA may be at a higher risk of developing complications, particularly pouchitis, stricture, and fistula; the ultimate and functional outcome of the pouch is similar to patients with FAP. Pouch failure, excision and revision were similar in the two groups.
Topics: Adenomatous Polyposis Coli; Adult; Anastomosis, Surgical; Colitis, Ulcerative; Colonic Pouches; Constriction, Pathologic; Female; Humans; Male; Postoperative Complications; Pouchitis; Proctocolectomy, Restorative; Retrospective Studies; Treatment Outcome
PubMed: 35357610
DOI: 10.1007/s10151-022-02617-w -
Colorectal Disease : the Official... Jan 2022Pouchitis is a clinically significant complication of ileal pouch-anal anastomosis with its prevalence varying in the literature. Pouchitis is thought to occur more... (Meta-Analysis)
Meta-Analysis Review
AIM
Pouchitis is a clinically significant complication of ileal pouch-anal anastomosis with its prevalence varying in the literature. Pouchitis is thought to occur more commonly in patients with ulcerative colitis (UC) than in patients with familial adenomatous polyposis (FAP). We conducted a systematic review with meta-analysis of all published literature to report the prevalence of pouchitis in all pouch patients as well as specifically in UC and FAP. We also investigated the prevalence of acute and chronic pouchitis in UC and FAP.
METHODS
A meta-analysis was conducted by searching the Embase, Embase Classic and PubMed databases between 1978 and 2021 exploring the prevalence of pouchitis in UC and FAP. We then performed a random effects model in order to find the pooled prevalence of pouchitis and used odds ratios to report differences in the prevalence of pouchitis in UC and FAP.
RESULTS
Fifty-nine full papers and conference abstracts were included in the meta-analysis comprising 18 117 patients with UC and 860 with FAP. The prevalence of pouchitis in UC was 0.32 (95% CI 0.27-0.37) and in FAP was found to be 0.06 (95% CI 0.03-0.15). The odds ratio of pouchitis in UC patients compared with FAP patients was 4.95 (95% CI 3.17-7.72, P < 0.0001).
CONCLUSIONS
Our findings support the consensus that the prevalence of pouchitis is higher in UC than in FAP. More significantly our findings suggest that the true prevalence of pouchitis is higher than commonly reported in the literature. This literature may help counsel patients prior to undergoing restorative proctocolectomy.
Topics: Adenomatous Polyposis Coli; Colitis, Ulcerative; Humans; Pouchitis; Prevalence; Proctocolectomy, Restorative
PubMed: 34800326
DOI: 10.1111/codi.15995 -
The American Journal of Gastroenterology Apr 2024Individuals with familial adenomatous polyposis (FAP) have an almost 20% lifetime risk of duodenal adenocarcinoma, currently the leading cause of death in FAP. The... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Individuals with familial adenomatous polyposis (FAP) have an almost 20% lifetime risk of duodenal adenocarcinoma, currently the leading cause of death in FAP. The Spigelman staging system provides guidance on the surveillance intervals and timing of prophylactic surgery. Still, its accuracy in predicting duodenal and papillary cancer development has not been systematically evaluated. We investigated the sensitivity and cancer risk of the Spigelman stages.
METHODS
We performed a systematic review on PubMed, MEDLINE, EMBASE, and Cochrane and used a random-effects model to pool effect sizes.
RESULTS
After removing duplicate entries, we screened 1,170 records and included 27 studies for quantitative analysis. Once duodenal polyposis reaches Spigelman stage IV, the risk of duodenal and papillary cancers increased to 25% (95% confidence interval [CI] 12%-45%). However, the sensitivity of Spigelman stage IV for these cancers was low (51%, 95% CI 42%-60%), especially for papillary adenocarcinoma (39%, 95% CI 16%-68%). We investigated the reasons behind these low values and observed that duodenal cancer risk factors included polyps >10 mm, polyp count >20, and polyps with high-grade dysplasia. Risk factors associated with papillary cancer included a papilla with high-grade dysplasia or >10 mm. The evidence on other risk factors was inconclusive.
DISCUSSION
The current Spigelman staging system had a low sensitivity for duodenal and papillary adenocarcinomas. Two Spigelman variables (duodenal villous histology and polyp count) and the lack of papilla-specific variables likely contributed to the low sensitivity values for duodenal and papillary cancers, respectively. While clinicians may be familiar with its current form, there is an urgent need to update it.
Topics: Humans; Adenomatous Polyposis Coli; Duodenum; Duodenal Neoplasms; Polyps; Risk Factors
PubMed: 38294150
DOI: 10.14309/ajg.0000000000002688 -
The British Journal of Surgery May 2024Hereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of...
Updated European guidelines for clinical management of familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), gastric adenocarcinoma, proximal polyposis of the stomach (GAPPS) and other rare adenomatous polyposis syndromes: a joint EHTG-ESCP revision.
BACKGROUND
Hereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of colorectal and other cancers.
METHODS
A team of 38 experts convened to update the 2008 European recommendations for the clinical management of patients with adenomatous polyposis syndromes. Additionally, other rare monogenic adenomatous polyposis syndromes were reviewed and added. Eighty-nine clinically relevant questions were answered after a systematic review of the existing literature with grading of the evidence according to Grading of Recommendations, Assessment, Development, and Evaluation methodology. Two levels of consensus were identified: consensus threshold (≥67% of voting guideline committee members voting either 'Strongly agree' or 'Agree' during the Delphi rounds) and high threshold (consensus ≥ 80%).
RESULTS
One hundred and forty statements reached a high level of consensus concerning the management of hereditary adenomatous polyposis syndromes.
CONCLUSION
These updated guidelines provide current, comprehensive, and evidence-based practical recommendations for the management of surveillance and treatment of familial adenomatous polyposis patients, encompassing additionally MUTYH-associated polyposis, gastric adenocarcinoma and proximal polyposis of the stomach and other recently identified polyposis syndromes based on pathogenic variants in other genes than APC or MUTYH. Due to the rarity of these diseases, patients should be managed at specialized centres.
Topics: Humans; Adenomatous Polyposis Coli; Stomach Neoplasms; Adenocarcinoma; DNA Glycosylases; Neoplastic Syndromes, Hereditary; Europe; Adenomatous Polyps; Polyps
PubMed: 38722804
DOI: 10.1093/bjs/znae070 -
Journal of Gastroenterology and... Jan 2024The purpose of this study was to assess evidence on the frequency of polyp surveillance colonoscopies performed earlier than the recommended follow-up intervals in... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIM
The purpose of this study was to assess evidence on the frequency of polyp surveillance colonoscopies performed earlier than the recommended follow-up intervals in clinical practice guidelines.
METHODS
A systematic review was performed based on electronic searches in PubMed and Embase. Research articles, letters to the editors, and review articles, published before April 2022, were included. Studies that focused on the intervals of polyp surveillance in adult populations were selected. The Risk Of Bias In Non-randomized Studies of Exposure (ROBINS-E) was used to assess the risk of bias. A meta-analysis was performed with Forest plots to illustrate the results.
RESULTS
In total, 16 studies, comprising 11 172 patients from Australia, Europe, and North America, were included for analysis. The quality of the studies was moderate. Overall, 38% (95% CI: 30-47%) of colonoscopies were undertaken earlier than their respective national clinical guidelines. In risk-stratified surveillance, 10 studies contained data relating to low-risk polyp surveillance intervals and 30% (95% CI: 29-31%) of colonoscopies were performed earlier than recommended. Eight studies contained data relating to intermediate-risk polyp surveillance and 15% (95% CI: 14-17%) of colonoscopies were performed earlier than recommended. One study showed that 6% (95% CI: 4-10%) of colonoscopies performed for high-risk polyp surveillance were performed earlier than recommended.
CONCLUSIONS
A significant proportion of polyp surveillance was performed earlier than the guidelines suggested. This provides evidence of the potential overuse of healthcare resources and the opportunity to improve hospital efficiency.
Topics: Humans; Colorectal Neoplasms; Adenomatous Polyps; Colonoscopy; Polyps; North America; Colonic Polyps
PubMed: 37967829
DOI: 10.1111/jgh.16397 -
Diseases of the Colon and Rectum Dec 2022In patients with ulcerative colitis or familial adenomatous polyposis who develop neoplasia or fail medical therapy and require colectomy, restorative proctocolectomy...
BACKGROUND
In patients with ulcerative colitis or familial adenomatous polyposis who develop neoplasia or fail medical therapy and require colectomy, restorative proctocolectomy with IPAA is often indicated. Although often well tolerated, IPAA can be complicated by cuffitis or inflammation of the remaining rectal cuff. Although much has been published on this subject, there is no clear and comprehensive synthesis of the literature regarding cuffitis.
METHODS
Our systematic literature review analyzes 34 articles to assess the frequency, cause, pathogenesis, diagnosis, classification, complications, and treatment of cuffitis.
RESULTS
Cuffitis occurs in an estimated 10.2% to 30.1% of pouch patients. Purported risk factors include rectal cuff length >2 cm, pouch-rectal anastomosis, stapled anastomosis, J-pouch configuration, 2- or 3-stage IPAA, preoperative Clostridium difficile infection, toxic megacolon, fulminant colitis, preoperative biologic use, medically refractory disease, immunomodulator/steroids use within 3 months of surgery, extraintestinal manifestations of IBD, and BMI <18.5 kg/m2 at the time of colectomy. Adverse consequences associated with cuffitis include decreased quality-of-life scores, increased risk for pouchitis, pouch failure, pouch excision, and pouch neoplasia.
CONCLUSIONS
Given the similarities between pouchitis and cuffitis, diagnosis and treatment of cuffitis should proceed according to the International Ileal Pouch Consortium guidelines. This review found that the majority of the current literature fails to distinguish between classic cuffitis (a form of reminant ulcerative proctitis) and nonclassic cuffitis (resulting from other causes). Further work is needed to distinguish the unique risk factors and endoscopic characteristics associated with each subtype, and further randomized clinical trials should be conducted to strengthen the evidence for treatment options.
Topics: Humans; Pouchitis; Proctocolectomy, Restorative; Colonic Pouches; Colitis, Ulcerative; Adenomatous Polyposis Coli
PubMed: 36399769
DOI: 10.1097/DCR.0000000000002593 -
Irish Journal of Medical Science Apr 2023Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically but more commonly are associated with genetic syndromes such as familial adenomatous polyposis (Sakorafas et al. in Surg Oncol 16(2):131-142, 2007) (FAP). Mutations in either the Wnt, β-catenin or APC genes are 'key' triggers for the development of these tumours (Howard and Pollock in Oncol Ther 4(1):57-72, 2016). Classically, these tumours do not metastasise; however, they are associated with significant morbidity and mortality due to their infiltrative pattern and/or local invasion. Historically, surgical resection was the cornerstone of treatment. There remains paucity of data regarding outcomes following the surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
OBJECTIVES
The aim of this review was to assess the current evidence for surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
METHODS
A systematic search of articles in PubMed, EMBASE and The Cochrane Library databases was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for the period from January 2000 to November 2020.
RESULTS
Twenty-three studies were included, of which, 749 patients had surgical resection (696 for primary and 53 for recurrent desmoids), 243 patients (18.8%) were medically managed and 353 patients (27.3%) underwent surveillance. Median follow-up was 51.4 months (range 1-372). Six-hundred and ninety-six of the 749 resections (92.9%) underwent primary desmoid resection, with the remaining 53 (7.1%) undergoing resection for recurrence. One-hundred and two surgically managed patients (19%) developed a (re)recurrence, with mesenteric involvement the commonest site for recurrence (55%). When comparing recurrence post-surgery to progression following medical therapy, there was a trend towards better outcomes with surgery, with 25% of surgical patients having a recurrence versus 50.5% having progression with medical therapy [OR 0.40 (95% CI 0.06-2.70), p = 0.35]. Major morbidity following surgery was 4.4% (n = 33) with 2% (n = 14) mortality within 30 days of resection.
CONCLUSION
The management of desmoids has considerable heterogeneity. Surgical resection for abdominal desmoids remains a valid treatment option in highly selective cases where negative margins can be obtained, with low major morbidity and/or mortality.
Topics: Humans; Fibromatosis, Aggressive; Fibromatosis, Abdominal; Adenomatous Polyposis Coli; Mutation; Colectomy
PubMed: 35445926
DOI: 10.1007/s11845-022-03008-8