Associations of long non-coding RNAs HOTAIR, LINC00951, POLR2E and HULC polymorphisms with the risk of esophageal and esophagogastric junction cancer in a western population: a case-control study.

BACKGROUND: The incidence of single-nucleotide-polymorphisms with malignant potential in esophageal cancer tissues has only been sparsely investigated in the west. Hence, we explored the contribution of four long non-coding RNAs' polymorphisms HOTAIR rs920778, LINC00951 rs11752942, POLR2E rs3787016 and HULC rs7763881 in esophageal cancer susceptibility. METHODS AND RESULTS: Formalin-fixed paraffin-embedded tissue specimens from 95 consecutive patients operated for esophageal/esophagogastric junction carcinoma during 25/03/2014-25/09/2018 were processed. Demographic data, histopathological parameters, surgical and oncological outcomes were collected. DNA findings of the abovementioned population were compared with 121 healthy community controls. Both populations were of European/Greek ancestry. Sixty-seven patients underwent Ivor Lewis/McKeown esophagectomy for either squamous cell esophageal carcinoma (N = 6) or esophageal/esophagogastric junction Siewert I or II adenocarcinoma (N = 61). Twenty-eight patients were subjected to extended total gastrectomy for esophagogastric junction Siewert III adenocarcinoma. Neither LINC00951 rs11752942 nor HULC rs7763881 polymorphisms were detected more frequently in esophageal cancer patients compared with healthy community subjects. A significantly higher presence of HOTAIR rs920778 TT genotype in esophagogastric junction Siewert I/II adenocarcinoma was identified. POLR2E rs3787016 C allele and CC genotypes were overrepresented in the control group, and when found in esophageal cancer carriers were associated with earlier disease stages, as well as with minor lymph node involvement and lesser metastatic potential. CONCLUSIONS: HOTAIR rs920778 may serve as a potential therapeutic suppression target, while POLR2E rs3787016 may represent a valuable biomarker to evaluate esophageal cancer predisposition and predict treatment response and prognosis. Clinical implications of these findings need to be verified with further prospective studies with larger sample-size.

Genetic Impact of HOTAIR, LINC00951, POLR2E and HULC Polymorphisms in Histopathological and Laboratory Prognostic Factors in Esophageal Cancer in the West: A Case-Control Study.

Long non-coding RNAs' HOTAIR rs920778, LINC00951 rs11752942, POLR2E rs3787016, and HULC rs7763881 are progressively reported having a close genetic affinity with esophageal carcinogenesis in the East. Nonetheless, their correlation with variables already endorsed as significant prognostic factors in terms of staging, guiding treatment and predicting recurrence, metastasis, and survival have yet to be explored. Herein, we investigated their prognostic value by correlating them with clinicopathological and laboratory prognostic markers in esophageal cancer in the West. Formalin-fixed paraffin-embedded tissue specimens from 95 consecutive patients operated on for esophageal cancer between 2014 and 2018 were compared with 121 healthy community controls. HULC was not detected differently in any of the cancer prognostic subgroups. LINC00951 was underrepresented in Ca19.9 elevated subgroup. HOTAIR was more frequent in both worse differentiation grade and positive Signet-Ring-Cell and Ca19.9 subgroups. POLR2E was identified less frequently in Adenocarcinoma, Signet-Ring-Cell, and Diffuse histologies, as well as in Perineural, Lymphovascular, and Perivascular Invasion positive, while it was overrepresented in CEA positive subgroup. These lncRNAs polymorphisms may hold great potential not only as future therapeutic agents but also as novel markers for predictive analysis of esophageal cancer risk, clinical outcome, and survival. Clinical implications of these findings need to be validated with prospective larger sample-size studies.

Identifying the role of preoperative oral/dental health care in post-esophagectomy pulmonary complications: a systematic review and meta-analysis.

Esophageal surgery has traditionally been associated with high morbidity rates. Despite the recent advances in the field of minimally invasive surgery and the introduction of enhanced recovery after surgery (ERAS) protocols, post-esophagectomy morbidity, especially that attributed to the respiratory system, remains a concern. In that respect, preoperative intensification of oral care or introduction of structured oral/dental hygiene regimens may lead to tangible postoperative benefits associated with reduced morbidity (respiratory or otherwise) and length of hospital stay. A systematic literature search of the Medline, Embase, Web of Knowledge and clinicaltrials.gov databases was undertaken for studies reporting use of preoperative oral/dental hygiene improvement regimens in patients scheduled to undergo esophagectomy for esophageal cancer. Meta-analysis was performed using a random-effects model. After screening 796 unique studies, seven were deemed eligible for inclusion in the meta-analysis. Pooled results indicated equivalent postoperative pneumonia rates in the oral pretreatment group and control groups (8.7 vs. 8.5%, respectively); however, the odds for developing pneumonia were reduced by 50% in the pretreatment group (odds ratio 0.5, 95% C.I. 0.37 to 0.69, P < 0.001). No statistically significant difference was detected in the anastomotic leak (odds ratio 0.93, 95% C.I. 0.38 to 2.24, P = 0.87) and length of stay outcomes (mean difference 0.63, 95% C.I. -3.22 to 4.47, P = 0.75). Oral/dental pretreatment reduces the odds for developing post-esophagectomy pneumonia. This finding should be cautiously interpreted given the significant limitations inherent in this meta-analysis. Further investigation via well-designed clinical trials is thus warranted before implementation in routine practice can be recommended.

Gastrectomy for Cancer: A 15-Year Analysis of Real-World Data from the University of Athens.

Background and Objectives: Encouraging data have been reported from referral centers following gastrointestinal cancer surgery. Our goal was to retrospectively review patient outcomes following gastrectomy for gastric or gastroesophageal junction (GEJ) cancer at a high-volume unit of the University of Athens. Methods: The enrollment period was from June 2003 to September 2018. Disease-free survival (DFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Cox proportional hazard models were constructed to identify variables independently associated with time-to-event outcomes. Results: A total of 205 patients were analyzed. R0 resection was achieved in 183 (89.3%) patients and was more likely to occur following neoadjuvant chemotherapy (p = 0.008). Recurrence developed in 46.6% of our cohort and the median disease-free survival was 31.2 months. On multivariate analysis, only staging (HR = 2.15; 95% CI: 1.06-4.36) was independently associated with increased risk of recurrence. All-cause mortality was 57.2% and the median time of death was 40.9 months. On multivariate regression, staging (HR: 1.35; 95% CI: 1.11-1.65) and recurrence (HR: 2.87; 95% CI: 1.32-6.22) predicted inferior prognosis. Conclusions: Gastrectomy at the University of Athens has yielded favorable outcomes for patients with GEJ cancer.

Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity.

Synovial sarcoma (SS) is a rare mesenchymal entity that represents 5-10% among soft tissue sarcomas (STS). Primary renal synovial sarcoma (PRSS) is an uncommon, rapidly growing tumor, with potential metastatic dissemination. The main prognostic factors of PRSS include tumor size and histologic grade, while translocation t (X; 18) (p11.2; q11.2) (fusion of SYT gene -chromosome 18- with SSX genes (1, 2 or 4)-chromosome X) is the most common pathognomonic sign. Aggressive surgical resection of the tumor along with concomitant regional lymphadenectomy is the treatment of choice for PRSS, while additional en bloc resection of the adjacent affected organs is often performed. To date, the role of preoperative or postoperative chemotherapy remains equivocal. The prognosis of patients with PRSS is poor, as the 5-year survival rate is only 20-30% and further deteriorates when a high mitotic activity is detected. Local recurrence even after complete R0 surgical excision remains the most frequent cause of death. The aim of this review was to meticulously discuss clinical features, histogenesis, and morphological and immunochemical findings of PRSS, while the role of current diagnostic and therapeutic management of this aggressive neoplasm was emphasized.

Safety Profile of Niraparib as Maintenance Therapy for Ovarian Cancer: A Systematic Review and Meta-Analysis.

BACKGROUND: Patients with epithelial ovarian cancer (EOC), treated with niraparib maintenance, present with haematological and gastrointestinal toxicities. Limited data exist on niraparib safety assessment. OBJECTIVE: To evaluate niraparib safety profile, as maintenance therapy, in women with platinum-sensitive EOC. METHODS: PubMed and Cochrane searches were carried out up to April 2021 for randomised controlled trials (RCTs) evaluating niraparib versus placebo in EOC patients with a response to platinum-based chemotherapy. Regarding the meta-analysis, for dichotomous data, the pooled risk ratio (RR) was calculated. RESULTS: A total of 1539 patients from three RCTs revealed that niraparib-treated patients are associated with a significantly higher risk of any grade of nausea (RR, 2.15; 95% CI, 1.86 to 2.48), fatigue (RR, 1.26; 95% CI, 1.05 to 1.52, p < 0.00001), anemia (RR, 6.86; 95% CI, 2.54 to 18.52, p = 0.0001), thrombocytopenia (RR, 7.02; 95% CI, 1.68 to 29.38, p < 0.00001), vomiting (RR, 2.51; 95% CI, 1.50 to 4.19, p = 0.0005), neutropenia (RR, 2.96; 95% CI, 1.13 to 7.73, p < 0.00001), headache (RR, 2.08; 95% CI, 1.57 to 2.74, p < 0.00001), constipation (RR, 2.10; 95% CI, 1.72 to 2.57, p < 0.00001) and insomnia (RR, 2.48; 95% CI, 1.52 to 2.89, p = 0.0003) when compared with placebo. For grade 3 or 4 adverse effects, significantly higher risk was only noted for fatigue (RR,6.25; 95% CI, 1.70 to 23.05, p = 0.006), anemia (RR, 16.23; 95% CI, 4.86 to 54.17, p < 0.00001), thrombocytopenia (RR, 35.12; 95% CI, 12.23 to 100.82, p < 0.00001) and neutropenia episodes (RR, 6.35; 95% CI, 2.08 to 19.39, p = 0.001) for those taking niraparib. Notably, incidents of adverse effects and discontinuation rates were substantially lower among patients treated with an individualised niraparib dose than those treated with the standard one. Efficacy was not reduced, and no treatment-related deaths occurred during the included trials. CONCLUSION: Niraparib is considered an effective and well-tolerated choice, with an improved safety profile, for the maintenance treatment of EOC patients.

Dealing With Burn Patients in War Zones.

OBJECTIVE: Treating burn patients in the battlefield is one of the biggest challenges that military doctors and medical personnel can face. Wound patterns have been changed over time due to the introduction of new weapons, and many different aspects play a major role in the management of those burns nowadays. There is a potential gap in care of burn patients in war zones. METHODS: A thorough literature search in PubMed, scientific journals, and Internet sites was conducted in regard to burn patients and trauma in war zones. RESULTS: It is crucial for military surgeons to be able to stabilize burn patients during wartime conflicts, especially those patients who suffer from extreme burn injuries, as specialized treatment should be given. Medical personnel should be aware of all medication types used, the ways to minimize the risk of bacterial infection, and the ways to keep the injured safe. CONCLUSIONS: Injured civilians with burn trauma in the field of battle are deserving care, and special recognition should be given to the non-governmental organizations (NGOs) that strive to ease human suffering in war zones. Proper management of burn patients in war zones is crucial, and military medical staff and NGOs can play a key role in that purpose.

Esophageal cancer surgery in Greece during the era of the financial crisis.

The aim of this study is to describe outcomes of esophageal cancer surgery in a quaternary upper gastrointestinal (GI) center in Athens during the era of the Greek financial crisis. We performed a retrospective analysis of patients that underwent esophagectomy for esophageal or gastroesophageal junction (GEJ) cancer at an upper GI unit of the University of Athens, during the period January 2004-June 2019. Time-to-event analyses were performed to explore trends in survival and recurrence. A total of 146 patients were identified. Nearly half of the patients (49.3%) underwent surgery during the last 4 years of the financial crisis (2015-2018). Mean age at the time of surgery was 62.3 ± 10.3 years, and patients did not present at older ages during the recession (P = 0.50). Most patients were stage III at the time of surgery both prior to the recession (35%) and during the financial crisis (39.8%, P = 0.17). Ivor-Lewis was the most commonly performed procedure (67.1%) across all eras (P = 0.06). Gastric conduit was the most common form of GI reconstruction (95.9%) following all types of surgery (P < 0.001). Pre-recession anastomoses were usually performed using a circular stapler (65%). Both during (88.1%) and following the recession (100%), the vast majority of anastomoses were hand-sewn. R0 resection was achieved in 142 (97.9%) patients. Anastomosis technique did not affect postoperative leak (P = 0.3) or morbidity rates (P = 0.1). Morbidity rates were not significantly different prior to (25%), during (46.9%), and after (62.5%) the financial crisis, P = 0.16. Utilization of neoadjuvant chemotherapy (26.9%, P = 0.90) or radiation (8.4%, P = 0.44) as well as adjuvant chemotherapy (54.8%, P = 0.85) and irradiation (13.7%, P = 0.49) was the same across all eras. Disease-free survival (DFS) and all-cause mortality rates were 41.2 and 47.3%, respectively. Median DFS and observed survival (OS) were 11.3 and 22.7 months, respectively. The financial crisis did not influence relapse (P = 0.17) and survival rates (P = 0.91). The establishment of capital controls also had no impact on recurrence (P = 0.18) and survival (P = 0.94). Austerity measures during the Greek financial crisis did not influence long-term esophageal cancer outcomes. Therefore, achieving international standards in esophagectomy may be possible in resource-limited countries when centralizing care.

Gastric ischemic preconditioning may reduce the incidence and severity of anastomotic leakage after οesophagectomy: a systematic review and meta-analysis.

Anastomotic leakage after esophagectomy is a severe and life-threatening complication. Gastric ischemic preconditioning is a strategy for the improvement of anastomotic healing. Aim of this systematic review and meta-analysis is to investigate the impact of gastric ischemic preconditioning on postoperative morbidity. A systematic literature search was performed to identify studies comparing patients undergoing gastric ischemic preconditioning before esophagectomy with nonpreconditioned patients. Meta-analysis was conducted for the overall incidence of anastomotic leakage, severe anastomotic leakage, anastomotic stricture, postoperative morbidity, and mortality. Mantel-Haenszel odds ratios (ORs) and 95% confidence intervals (CIs) were calculated. Subgroup analyses were performed concerning preconditioning technique, the interval between preconditioning and surgery and the extent of preconditioning. Fifteen cohort studies were identified. Gastric preconditioning was associated with reduced overall incidence of anastomotic leakage (OR 0.73; 95% CI, 0.53-1.0; P = 0.050) and severe anastomotic leakage (OR 0.27; 95% CI, 0.14-0.50; P < 0.010), but not with anastomotic stricture (OR 1.18; 95% CI 0.38 to 3.66; P = 0.780), major postoperative morbidity (OR 1.03; 95% CI 0.45 to 2.36; P = 0.940) or mortality (OR 0.69; 95% CI 0.39 to 1,23; P = 0.210). Subgroup analyses did not identify any differences between embolization and ligation while increasing the interval between preconditioning and esophagectomy as well as the extent of preconditioning might be beneficial. Gastric ischemic preconditioning may be associated with a reduced incidence of overall and severe anastomotic leakage. Randomized studies are necessary to further evaluate its impact on leakage, refine the technique and define patient populations that will benefit the most.

Glycoprotein non-metastatic melanoma B expression after hepatic ischemia reperfusion and the effect of silibinin.

BACKGROUND: Glycoprotein non-metastatic melanoma B (GPNMB) is a transmembrane glycoprotein with various roles in inflammation regulation, tissue remodeling and oncogenesis. Clinical situations implicating alterations in its expression include ischemic injury, cirrhosis and fatty liver disease amongst other. We examine its expression in hepatic and renal tissue following hepatic ischemia-reperfusion (I/R) in a rat model, with and without intravenous silibinin administration, as a silibinin-hydroxypropyl-ß-cyclodextrin lyophilized complex (SLB-HP-ß-CD). METHODS: Sixty-three Wistar rats were divided into 3 groups: sham group (virtual intervention; 7 animals), control (C) group (45 min of ischemia, followed by reperfusion and euthanasia at 60, 120, 180 and 240 min; 28 animals equally divided), and silibinin (Si) group (45 min of ischemia, intravenous administration of SLB-HP-ß-CD, reperfusion and euthanasia at the same time points; 28 animals equally divided). GPNMB expression was examined in liver and kidney tissue. RESULTS: GPNMB expression was significantly increased following hepatic I/R in the control group, in kidney tissue, in a time dependent manner. In the silibinin group, GPNMB expression significantly decreased with time compared to the control group in both liver and kidney tissue (P<0.05). CONCLUSIONS: Hepatic I/R causes increase of GPNMB levels both in liver and kidney tissues, which may reflect tissue injury. Silibinin seems to act protectively on both liver and kidney, and can be potentially used as a therapeutic approach against hepatic I/R injury.

Pseudoachalasia: a systematic review of the literature.

Pseudoachalasia, also known as secondary achalasia, is a clinical condition mimicking idiopathic achalasia but most commonly caused by malignant tumors of gastroesophageal junction (GEJ). Our aim was to systematically review and present all available data on demographics, clinical features, and diagnostic modalities involved in patients with pseudoachalasia. A systematic search of literature published during the period 1978-2019 was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (end-of-search date: June 25th, 2019). Two independent reviewers extracted data with regards of study design, interventions, participants, and outcomes. Thirty-five studies met our inclusion criteria and were selected in the present review. Overall, 140 patients with pseudoachalasia were identified, of whom 83 were males. Mean patient age was 60.13 years and the mean weight loss was 13.91 kg. A total of 33 (23.6%) patients were wrongly 'treated' at first for achalasia. The most common presenting symptoms were dysphagia, food regurgitation, and weight loss. The median time from symptoms' onset to hospital admission was 5 months. Most common etiology was gastric cancer (19%). Diagnostic modalities included manometry, barium esophagram, endoscopy, and computed tomography (CT). Pseudoachalasia is a serious medical condition that is difficult to be distinguished from primary achalasia. Clinical feature assessment along with the correct interpretation of diagnostic tests is nowadays essential steps to differentiate pseudoachalasia from idiopathic achalasia.

Hybrid Laparoscopic and Endoscopic Partial Gastrectomy for Ulcerated GIST: Surgical Technique with Video.

INTRODUCTION: Gastrointestinal stromal tumors of the gastric cardia pose several technical difficulties to their resection because they are difficult to access, and they must be removed respecting oncological principles, and due to their proximity to the gastroesophageal junction, excessive removal of gastric tissue may cause deformation with gastric dysfunction, or gastroesophageal reflux. METHOD: Hybrid laparoscopic endoscopic partial gastrectomy is a technique that avoids these problems making use of three principles. First the tumor is pinpointed, marked, and resected under combined laparoscopic and endoscopic control to assure that it is completely resected without compromising the structure of the gastroesophageal junction. Second, a dissection of the abdominal esophagus is performed as during a Nissen fundoplication in order to increase the distance between the tumor and the gastroesophageal junction. Third the lesser curvature of the stomach is dissected, and the terminal branches of the left gastric vessels are controlled from the gastric incisura to the gastroesophageal junction exposing the lesser curvature and the posterior wall of the stomach. The tumor is resected under endoscopic control, tangentially with an endoscopic stapler applied parallel to the lesser curvature similarly to a sleeve partial gastrectomy. RESULTS: No abdominal drain is used, and a nasogastric tube is left in place for the first 24 h after surgery. The patient receives fluid diet on the first postoperative day, is discharged on the second postoperative day, and is followed up in the outpatient department for a week. CONCLUSION: In this technical paper, the procedure is described in detail and demonstrated with video.

Staged esophagectomy: surgical legacy or a bailout option?

Staged esophagectomy was developed in the mid-twentieth century in an attempt to reduce high rates of postoperative morbidity and mortality. Nowadays, the operation has almost been abandoned due to its significant disadvantages, especially the need for multiple surgeries, inability of patients to feed between operations, and morbidity of esophageal stoma. However, staged esophagectomy is still occasionally useful for very high-risk patients and in particular cases, for example multiple cancers of the aerodigestive tract and emergent esophagectomy. Staged esophagectomy is based on the division of surgical stress into two operations, which gives the patient time to recover before final restoration. Gastric tube ischemic preparation may be a more important mechanism in staged esophagectomy. This approach may survive and expand with the application of ischemic gastric pre-conditioning through embolization or laparoscopic ligation of the gastric arteries, which is a less explored and promising technique.

New insights into irritable bowel syndrome: from pathophysiology to treatment.

Irritable bowel syndrome (IBS) is the most common reason to visit a gastroenterologist. IBS was believed to be a functional disease, but many possible pathophysiologic mechanisms can now explain the symptoms. IBS patients are classified into subtypes according to their predominant bowel habit, based on the Rome IV criteria. These include diarrhea-predominant and constipation-predominant IBS, as well as the mixed type, a combination of the two. Usually, IBS treatment is based on the predominant symptoms, with many options for each subtype. A new promising treatment option, fecal microbiota transplantation, seems to have beneficial effects on IBS. However, treating the pathophysiological causative agent responsible for the symptoms is an emerging approach. Therefore, before the appropriate therapeutic option is chosen for treating IBS, a clinical evaluation of its pathophysiology should be performed.

Synchronous resection of esophageal cancer and other organ malignancies: A systematic review.

BACKGROUND: Neoplasms arising in the esophagus may coexist with other solid organ or gastrointestinal tract neoplasms in 6% to 15% of patients. Resection of both tumors synchronously or in a staged procedure provides the best chances for long-term survival. Synchronous resection of both esophageal and second primary malignancy may be feasible in a subset of patients; however, literature on this topic remains rather scarce. AIM: To analyze the operative techniques employed in esophageal resections combined with gastric, pancreatic, lung, colorectal, kidney and liver resections and define postoperative outcomes in each case. METHODS: We conducted a systematic review according to PRISMA guidelines. We searched the Medline database for cases of patients with esophageal tumors coexisting with a second primary tumor located in another organ that underwent synchronous resection of both neoplasms. All English language articles deemed eligible for inclusion were accessed in full text. Exclusion criteria included: (1) Hematological malignancies; (2) Head/neck/pharyngeal neoplasms; (3) Second primary neoplasms in the esophagus or the gastroesophageal junction; (4) Second primary neoplasms not surgically excised; and (5) Preclinical studies. Data regarding the operative strategy employed, perioperative outcomes and long-term outcomes were extracted and analyzed using descriptive statistics. RESULTS: The systematic literature search yielded 23 eligible studies incorporating a total of 117 patients. Of these patients, 71% had a second primary neoplasm in the stomach. Those who underwent total gastrectomy had a reconstruction using either a colonic (n = 23) or a jejunal (n = 3) conduit while for those who underwent gastric preserving resections (i.e., non-anatomic/wedge/distal gastrectomies) a conventional gastric pull-up was employed. Likewise, in cases of patients who underwent esophagectomy combined with pancreaticoduodenectomy (15% of the cohort), the decision to preserve part of the stomach or not dictated the reconstruction method (whether by a gastric pull-up or a colonic/jejunal limb). For the remaining patients with coexisting lung/colorectal/kidney/liver neoplasms (14% of the entire patient population) the types of resections and operative techniques employed were identical to those used when treating each malignancy separately. CONCLUSION: Despite the poor quality of available evidence and the great interstudy heterogeneity, combined procedures may be feasible with acceptable safety and satisfactory oncologic outcomes on individual basis.

The profile of patients receiving public and private surgical services in Greece during the economic crisis: a comparative study.

BACKGROUND: International experience has shown that deterioration of healthcare services is a common consequence of socio-economic crises. Exact mechanism of this deterioration varies with respect to particularities of each healthcare system, government and administrative policies and local epidemiological conditions. The aim of the present study was to evaluate the effect of Greek economic crisis on the profile and the satisfaction rates of patients seeking surgical services in public and private hospitals. METHODS: A questionnaire-based survey concerning healthcare quality and patients' satisfaction was conducted at a private and a public (university) hospital. Patient demographics alongside with patient satisfaction before and after treatment were quantified and compared. RESULTS: Significant differences between private and public sector patients were found concerning nationality, socio-economic status and medical conditions. Private sector patients are younger, of a higher socio-economic status and admitted for elective rather than urgent medical conditions. Patient expectations before treatment are lower for public sector concerning a variety of markers but patient satisfaction is similar. CONCLUSIONS: Even in the years of financial crisis, Greek patients seem to be satisfied by the quality of the healthcare services in both public and private hospitals. Despite the limitations of our study regarding the selection of the population, we believe that the findings might generate more meticulous research on the field hoping that juxtaposed discussions will sensitize policy makers.

Arc of Bühler: the surgical significance of a rare anatomical variation.

PURPOSE: The arc of Bühler, an anastomotic vessel between celiac artery and superior mesenteric artery, is a rare anatomic variation. Various radiologic and surgical procedures can be affected by its existence. We aim to review all available information and identify possible clinical implications. METHODS: A systematic review was conducted in accordance to the Preferred Reporting Items for Systematic reviews and Meta-Analysis (PRISMA) guidelines. The following terms were utilized in various combinations: "Bühler", "arc of Bühler", "visceral aneurysm", "pancreaticoduodenal arcades". RESULTS: Only 53 cases have been described until today in the literature. The arc of Bühler enhances collateral circulation between celiac artery and superior mesenteric artery alongside with pancreaticoduodenal arcades and dorsal pancreatic artery. Computerized tomography and angiography are the main studies used for its detection and evaluation. Aneurysms of Bühler's arc have been rarely described and seem to share common pathophysiological mechanisms with aneurysms of the pancreaticoduodenal arcades. CONCLUSIONS: Various radiologic and surgical procedures such as embolization or pancreaticoduodenectomy are potentially affected by its existence.