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Background: The prognosis for liver cancer (LC) is dismal. Researchers recently discovered cuproptosis, a novel form of controlled cell death whose expression in LC and prognosis are unclear. This study reveals a gene signature to predict LC prognosis., Methods: RNA and clinical data for 371 LC patients were obtained from The Cancer Genome Atlas (TCGA). Differentially expressed genes (DEGs) were identified by comparing cancerous and normal samples. Genes linked to overall survival (OS) were found using univariate Cox regression and least absolute shrinkage and selection operator (LASSO). The gene signature was validated across all patients. Gene expression and clinical traits were analyzed, and Kaplan-Meier (KM) curves were generated for high- and low-risk groups. DEGs were used for Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG), immune infiltration, and drug prediction analyses. DLAT 's functions were assessed using real-time polymerase chain reaction (RT-PCR), transwell invasion, Cell Counting Kit-8 (CCK-8), colony formation, and drug resistance assays., Results: A total of 12 cuproptosis regulators were discovered in LC and normal liver tissues. A 3-gene signature based on LASSO Cox regression was utilized to categorize TCGA LC patients into low- and high-risk categories. Low-risk patients exhibited better survival than high-risk patients (P<0.05). Tumor grade, stage, and T stage differed between high- and low-risk groups. Long-term prognosis was well predicted by male subgroup survival studies. We predicted LC patient survival using sex, tumor grade, tumor stage, and risk score. Functional enrichment showed that extracellular matrix (ECM) architecture, channel function, and tumor-associated pathways were enriched in LC, suggesting that cancer related functions were collected. Immune microenvironment inhibition was found in the high-risk group suggesting that immunosuppression was closely related. We also discovered five small molecules that could be potentially useful for LC treatment. DLAT was discovered to promote the migration and proliferation of LC cells and is connected to drug resistance as a prognostic marker., Conclusions: Cuproptosis-related genes contribute to tumor development and can aid the prediction of LC patient prognosis. DLAT is a potential LC prognostic and therapeutic target., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jgo.amegroups.com/article/view/10.21037/jgo-24-609/coif). The authors have no conflicts of interest to declare., (2024 AME Publishing Company. All rights reserved.)

Artificial intelligence (AI) has a wide and increasing range of applications across various sectors. In medicine, AI has already made an impact in numerous fields, rapidly transforming healthcare delivery through its growing applications in diagnosis, treatment and overall patient care. Equally, AI is swiftly and essentially transforming the landscape of kidney transplantation (KT), offering innovative solutions for longstanding problems that have eluded resolution through traditional approaches outside its spectrum. The purpose of this review is to explore the present and future applications of artificial intelligence in KT, with a focus on pre-transplant evaluation, surgical assistance, outcomes and post-transplant care. We discuss its great potential and the inevitable limitations that accompany these technologies. We conclude that by fostering collaboration between AI technologies and medical practitioners, we can pave the way for a future where advanced, personalised care becomes the standard in KT and beyond.

Objective: To compare minimally invasive and open pancreatoduodenectomy in different subtypes of ampullary adenocarcinoma., Summary Background Data: Ampullary adenocarcinoma (AAC) is widely seen as the best indication for minimally invasive pancreatoduodenectomy (MIPD) due to the lack of vascular involvement and dilated bile and pancreatic duct. However, it is unknown whether outcomes of MIPD for AAC differ between the pancreatobiliary (AAC-PB) and intestinal (AAC-IT) subtypes as large studies are lacking., Methods: This is an international cohort study, encompassing 27 centers from 12 countries. Outcome of MIPD and open pancreatoduodenectomy (OPD) were compared in patients with AAC-IT and AAC-PB. Primary end points were R1 rate, lymph node yield, and 5-year overall survival (5yOS)., Results: Overall, 1187 patients after MIPD for AAC were included, of whom 572 with AAC-IT (62 MIPD, 510 OPD) and 615 with AAC-PB (41 MIPD and 574 OPD). The rate of R1 resection was not significantly different between MIPD and OPD for both AAC-IT (3.4% vs 6.9%, P=0,425) and AAC-PB (9.8% vs 14.9%, P=0,625). AAC-IT, more lymph nodes were resected with MIPD group (19 vs 16, P=0.007), compared to OPD. The 5y-OS did not differ after MIPD and OPD for both AAC-IT (56.8% vs 59.5%, P=0.827 and AAC-PB (52.5% vs 44.4%, P=0.357). The rates of surgical complication between MIPD and OPD did not differ between AmpIT and AmpPB., Discussion: This international multicenter study found no differences in outcomes between MIPD and OPD for AAC-IT and AAC-PB. MIPD and OPD demonstrated comparable outcomes in oncological resection, survival and surgical outcomes for both subtypes of AAC., Competing Interests: Conflicts of interests: Mario Serradilla-Martín received research funding from Baxter S.L.. Patrick Pessaux is co-founder of Virtualisurg; Geert Kazemier is Chairman of the Advisory Board of Renovaro Inc.; Marc G. Besselink has received grants from Intuitive for the LEARNBOT European robot Whipple training program, the DIPLOMA-2 trial, and the E-MIPS quality registry, from Medtronic for the investigator-initiated DIPLOMA trial and from Ethicon for the PANDORA trial and the E-MIPS quality registry; Mohammed Abu Hilal received funding from Associazione Italiana per la Ricerca sul Cancro (AIRC); All remaining authors claim no conflicts of interests and no disclosures., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Cancer arising in the periampullary region can be anatomically classified in pancreatic ductal adenocarcinoma (PDAC), distal cholangiocarcinoma (dCCA), duodenal adenocarcinoma (DAC), and ampullary carcinoma. Based on histopathology, ampullary carcinoma is currently subdivided in intestinal (AmpIT), pancreatobiliary (AmpPB), and mixed subtypes. Despite close anatomical resemblance, it is unclear how ampullary subtypes relate to the remaining periampullary cancers in tumor characteristics and behavior., Methods: This international cohort study included patients after curative intent resection for periampullary cancer retrieved from 44 centers (from Europe, United States, Asia, Australia, and Canada) between 2010 and 2021. Preoperative CA19-9, pathology outcomes and 8-year overall survival were compared between DAC, AmpIT, AmpPB, dCCA, and PDAC., Results: Overall, 3809 patients were analyzed, including 348 DAC, 774 AmpIT, 848 AmpPB, 1,036 dCCA, and 803 PDAC. The highest 8-year overall survival was found in patients with AmpIT and DAC (49.8% and 47.9%), followed by AmpPB (34.9%, P < 0.001), dCCA (26.4%, P  =  0.020), and finally PDAC (12.9%, P < 0.001). A better survival was correlated with lower CA19-9 levels but not with tumor size, as DAC lesions showed the largest size., Conclusions: Despite close anatomic relations of the five periampullary cancers, this study revealed differences in preoperative blood markers, pathology, and long-term survival. More tumor characteristics are shared between DAC and AmpIT and between AmpPB and dCCA than between the two ampullary subtypes. Instead of using collective definitions for "periampullary cancers" or anatomical classification, this study emphasizes the importance of individual evaluation of each histopathological subtype with the ampullary subtypes as individual entities in future studies., (© 2024. Society of Surgical Oncology.)

Background: Diabetes mellitus (DM) has a complex relationship with pancreatic cancer. This study examines the impact of preoperative DM, both recent-onset and pre-existing, on long-term outcomes following pancreatoduodenectomy (PD) for pancreatic ductal adenocarcinoma (PDAC)., Methods: Data were extracted from the Recurrence After Whipple's (RAW) study, a multi-centre cohort of PD for pancreatic head malignancy (2012-2015). Recurrence and five-year survival rates of patients with DM were compared to those without, and subgroup analysis performed to compare patients with recent-onset DM (less than one year) to patients with established DM., Results: Out of 758 patients included, 187 (24.7%) had DM, of whom, 47 of the 187 (25.1%) had recent-onset DM. There was no difference in the rate of postoperative pancreatic fistula (DM: 5.9% vs no DM 9.8%; p = 0.11), five-year survival (DM: 24.1% vs no DM: 22.9%; p = 0.77) or five-year recurrence (DM: 71.7% vs no DM: 67.4%; p = 0.32). There was also no difference between patients with recent-onset DM and patients with established DM in postoperative outcomes, recurrence, or survival., Conclusion: We found no difference in five-year recurrence and survival between diabetic patients and those without diabetes. Patients with pre-existing DM should be evaluated for PD on a comparable basis to non-diabetic patients., (Copyright © 2024 International Hepato-Pancreato-Biliary Association Inc. All rights reserved.)

Background: Despite differences in tumour behaviour and characteristics between duodenal adenocarcinoma (DAC), the intestinal (AmpIT) and pancreatobiliary (AmpPB) subtype of ampullary adenocarcinoma and distal cholangiocarcinoma (dCCA), the effect of adjuvant chemotherapy (ACT) on these cancers, as well as the optimal ACT regimen, has not been comprehensively assessed. This study aims to assess the influence of tailored ACT on DAC, dCCA, AmpIT, and AmpPB., Patients and Methods: Patients after pancreatoduodenectomy for non-pancreatic periampullary adenocarcinoma were identified and collected from 36 tertiary centres between 2010 - 2021. Per non-pancreatic periampullary tumour type, the effect of adjuvant chemotherapy and the main relevant regimens of adjuvant chemotherapy were compared. The primary outcome was overall survival (OS)., Results: The study included a total of 2866 patients with DAC (n = 330), AmpIT (n = 765), AmpPB (n = 819), and dCCA (n = 952). Among them, 1329 received ACT, and 1537 did not. ACT was associated with significant improvement in OS for AmpPB (P = 0.004) and dCCA (P < 0.001). Moreover, for patients with dCCA, capecitabine mono ACT provided the greatest OS benefit compared to gemcitabine (P = 0.004) and gemcitabine - cisplatin (P = 0.001). For patients with AmpPB, no superior ACT regime was found (P > 0.226). ACT was not associated with improved OS for DAC and AmpIT (P = 0.113 and P = 0.445, respectively)., Discussion: Patients with resected AmpPB and dCCA appear to benefit from ACT. While the optimal ACT for AmpPB remains undetermined, it appears that dCCA shows the most favourable response to capecitabine monotherapy. Tailored adjuvant treatments are essential for enhancing prognosis across all four non-pancreatic periampullary adenocarcinomas., (© 2024. The Author(s), under exclusive licence to Springer Nature Limited.)

Background: Standard lymphadenectomy for pancreatoduodenectomy is defined for pancreatic ductal adenocarcinoma and adopted for patients with non-pancreatic periampullary cancer (NPPC), ampullary adenocarcinoma (AAC), distal cholangiocarcinoma (dCCA), or duodenal adenocarcinoma (DAC). This study aimed to compare the patterns of lymph node metastases among the different NPPCs in a large series and in a systematic review to guide the discussion on surgical lymphadenectomy and pathology assessment., Methods: This retrospective cohort study included patients after pancreatoduodenectomy for NPPC with at least one lymph node metastasis (2010-2021) from 24 centers in nine countries. The primary outcome was identification of lymph node stations affected in case of a lymph node metastasis per NPPC. A separate systematic review included studies on lymph node metastases patterns of AAC, dCCA, and DAC., Results: The study included 2367 patients, of whom 1535 had AAC, 616 had dCCA, and 216 had DAC. More patients with pancreatobiliary type AAC had one or more lymph node metastasis (67.2% vs 44.8%; P < 0.001) compared with intestinal-type, but no differences in metastasis pattern were observed. Stations 13 and 17 were most frequently involved (95%, 94%, and 90%). Whereas dCCA metastasized more frequently to station 12 (13.0% vs 6.4% and 7.0%, P = 0.005), DAC metastasized more frequently to stations 6 (5.0% vs 0% and 2.7%; P < 0.001) and 14 (17.0% vs 8.4% and 11.7%, P = 0.015)., Conclusion: This study is the first to comprehensively demonstrate the differences and similarities in lymph node metastases spread among NPPCs, to identify the existing research gaps, and to underscore the importance of standardized lymphadenectomy and pathologic assessment for AAC, dCCA, and DAC., (© 2024. The Author(s).)

Introduction: Patients undergoing pancreaticoduodenectomy for distal cholangiocarcinoma (dCCA) often develop cancer recurrence. Establishing timing, patterns and risk factors for recurrence may help inform surveillance protocol strategies or select patients who could benefit from additional systemic or locoregional therapies. This multicentre retrospective cohort study aimed to determine timing, patterns, and predictive factors of recurrence following pancreaticoduodenectomy for dCCA., Materials and Methods: Patients who underwent pancreaticoduodenectomy for dCCA between June 2012 and May 2015 with five years of follow-up were included. The primary outcome was recurrence pattern (none, local-only, distant-only or mixed local/distant). Data were collected on comorbidities, investigations, operation details, complications, histology, adjuvant and palliative therapies, recurrence-free and overall survival. Univariable tests and regression analyses investigated factors associated with recurrence., Results: In the cohort of 198 patients, 129 (65%) developed recurrence: 30 (15%) developed local-only recurrence, 44 (22%) developed distant-only recurrence and 55 (28%) developed mixed pattern recurrence. The most common recurrence sites were local (49%), liver (24%) and lung (11%). 94% of patients who developed recurrence did so within three years of surgery. Predictors of recurrence on univariable analysis were cancer stage, R1 resection, lymph node metastases, perineural invasion, microvascular invasion and lymphatic invasion. Predictors of recurrence on multivariable analysis were female sex, venous resection, advancing histological stage and lymphatic invasion., Conclusion: Two thirds of patients have cancer recurrence following pancreaticoduodenectomy for dCCA, and most recur within three years of surgery. The commonest sites of recurrence are the pancreatic bed, liver and lung. Multiple histological features are associated with recurrence., (© 2024 Published by Elsevier Ltd.)

The term hepatolithiasis describes the presence of biliary stones within the intrahepatic bile ducts, above the hilar confluence of the hepatic ducts. The disease is more prevalent in Asia, mainly owing to socioeconomic and dietary factors, as well as the prevalence of biliary parasites. In the last century, owing to migration, its global incidence has increased. The main pathophysiological mechanisms involve cholangitis, bile infection and biliary strictures, creating a self-sustaining cycle that perpetuates the disease, frequently characterised by recurrent episodes of bacterial infection referred to as syndrome of "recurrent pyogenic cholangitis". Furthermore, long-standing hepatolithiasis is a known risk factor for development of intrahepatic cholangiocarcinoma. Various classifications have aimed at providing useful insight of clinically relevant aspects and guidance for treatment. The management of symptomatic patients and those with complications can be complex, and relies upon a multidisciplinary team of hepatologists, endoscopists, interventional radiologists and hepatobiliary surgeons, with the main goal being to offer relief from the clinical presentations and prevent the development of more serious complications. This comprehensive review provides insight on various aspects of hepatolithiasis, with a focus on epidemiology, new evidence on pathophysiology, most important clinical aspects, different classification systems and contemporary management., Competing Interests: Conflict-of-interest statement: The authors declare no conflict of interests for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Backgrounds/aims: After pancreatoduodenectomy (PD), an early oral diet is recommended; however, the postoperative nutritional management of PD patients is known to be highly variable, with some centers still routinely providing parenteral nutrition (PN). Some patients who receive PN experience clinically significant complications, underscoring its judicious use. Using a large cohort, this study aimed to determine the proportion of PD patients who received postoperative nutritional support (NS), describe the nature of this support, and investigate whether receiving PN correlated with adverse perioperative outcomes., Methods: Data were extracted from the Recurrence After Whipple's study, a retrospective multicenter study of PD outcomes., Results: In total, 1,323 patients (89%) had data on their postoperative NS status available. Of these, 45% received postoperative NS, which was "enteral only," "parenteral only," and "enteral and parenteral" in 44%, 35%, and 21% of cases, respectively. Body mass index < 18.5 kg/m 2 ( p = 0.03), absence of preoperative biliary stenting ( p = 0.009), and serum albumin < 36 g/L ( p = 0.009) all correlated with receiving postoperative NS. Among those who did not develop a serious postoperative complication, i.e., those who had a relatively uneventful recovery, 20% received PN., Conclusions: A considerable number of patients who had an uneventful recovery received PN. PN is not without risk, and should be reserved for those who are unable to take an oral diet. PD patients should undergo pre- and postoperative assessment by nutrition professionals to ensure they are managed appropriately, and to optimize perioperative outcomes.

This international multicenter cohort study included 30 centers. Patients with duodenal adenocarcinoma (DAC), intestinal-type (AmpIT) and pancreatobiliary-type (AmpPB) ampullary adenocarcinoma, distal cholangiocarcinoma (dCCA), and pancreatic ductal adenocarcinoma (PDAC) were included. The primary outcome was 30-day or in-hospital mortality, and secondary outcomes were major morbidity (Clavien-Dindo 3b≥), clinically relevant post-operative pancreatic fistula (CR-POPF), and length of hospital stay (LOS). Results: Overall, 3622 patients were included in the study (370 DAC, 811 AmpIT, 895 AmpPB, 1083 dCCA, and 463 PDAC). Mortality rates were comparable between DAC, AmpIT, AmpPB, and dCCA (ranging from 3.7% to 5.9%), while lower for PDAC (1.5%, p = 0.013). Major morbidity rate was the lowest in PDAC (4.4%) and the highest for DAC (19.9%, p < 0.001). The highest rates of CR-POPF were observed in DAC (27.3%), AmpIT (25.5%), and dCCA (27.6%), which were significantly higher compared to AmpPB (18.5%, p = 0.001) and PDAC (8.3%, p < 0.001). The shortest LOS was found in PDAC (11 d vs. 14-15 d, p < 0.001). Discussion: In conclusion, this study shows significant variations in perioperative mortality, post-operative complications, and hospital stay among different periampullary cancers, and between the ampullary subtypes. Further research should assess the biological characteristics and tissue reactions associated with each type of periampullary cancer, including subtypes, in order to improve patient management and personalized treatment.

Backgrounds/aims: Pancreatoduodenectomy (PD) is recommended in fit patients with a carcinoma (PDAC) of the pancreatic head, and a delayed resection may affect survival. This study aimed to correlate the time from staging to PD with long-term survival, and study the impact of preoperative investigations (if any) on the timing of surgery., Methods: Data were extracted from the Recurrence After Whipple's (RAW) study, a multicentre retrospective study of PD outcomes. Only PDAC patients who underwent an upfront resection were included. Patients who received neoadjuvant chemo-/radiotherapy were excluded. Group A (PD within 28 days of most recent preoperative computed tomography [CT]) was compared to group B (> 28 days)., Results: A total of 595 patents were included. Compared to group A (median CT-PD time: 12.5 days, interquartile range: 6-21), group B (49 days, 39-64.5) had similar one-year survival (73% vs. 75%, p = 0.6), five-year survival (23% vs. 21%, p = 0.6) and median time-todeath (17 vs. 18 months, p = 0.8). Staging laparoscopy (43 vs. 29.5 days, p = 0.009) and preoperative biliary stenting (39 vs. 20 days, p < 0.001) were associated with a delay to PD, but magnetic resonance imaging (32 vs. 32 days, p = 0.5), positron emission tomography (40 vs. 31 days, p > 0.99) and endoscopic ultrasonography (28 vs. 32 days, p > 0.99) were not., Conclusions: Although a treatment delay may give rise to patient anxiety, our findings would suggest this does not correlate with worse survival. A delay may be necessary to obtain further information and minimize the number of PD patients diagnosed with early disease recurrence.

Background: The global incidence of intrahepatic cholangiocarcinoma (ICCA) is soaring. Due to often delayed presentation, only a narrow spectrum of the disease is usually surgically resectable. To more accurately stage the disease, reduce recurrence, and improve overall survival, surgical teams are increasingly performing intraoperative lymph node dissection (LND) as well. This procedure has its associated morbidity, while there is no consensus or formal guidelines on its role in this setting. Hence, there is a need to better delineate the evidence for performing LND alongside surgical resection of the ICCA., Aim: To perform a systematic review and meta-analysis on the role of LND in improving prognostication and survival post-resection of ICCA., Methods: We performed a systematic literature search using Pubmed, Medline, Embase, and the Cochrane Library, for all studies involving LND, ICCA, and surgical resection using several keywords, Medical Subject Headings (MeSH) tags, and appropriate synonyms. All clinical studies comparing curative intent resection of ICCA with LND vs resection without LND were included, while single-arm case series, studies with insufficient data, and duplicates were excluded. We included all English-language studies from the different academic databases up till early December 2022. The primary outcome measures were set for overall survival (OS) and disease-free survival (DFS)., Results: This systematic review and meta-analysis included 15 studies that fulfilled the selection criteria comprising 11413 patients with surgically-resectable ICCA, of whom 6424 (56.3%) underwent hepatectomy with LND while the remainder underwent hepatectomy only. In patients who underwent LND, on average, 27.7% of the resected lymph nodes were positive for metastatic disease. Overall, the results showed that performing LND did not significantly improve OS or DFS. However, the effect of LND on OS showed a degree of variability by geographical region, in Eastern and Western countries. As LND is increasingly being performed, further time-based analysis was undertaken to identify time-dependent changes in the role of LND. An increasing adoption of LND was not associated with improved OS. Furthermore, no roles were identified for neoadjuvant/adjuvant chemotherapy or increasing lymph node retrieval in improving OS either., Conclusion: LND might aid in staging, prognosticating, and deciding further management of resected ICCA, but does not improve OS and DFS and is unsuitable for high-risk patients unlikely to benefit from further treatments., Competing Interests: Conflict-of-interest statement: The authors declare no conflict of interests for this article., (©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: Pancreatoduodenectomy (PD) is associated with significant postoperative morbidity. Surgeons should have a sound understanding of the potential complications for consenting and benchmarking purposes. Furthermore, preoperative identification of high-risk patients can guide patient selection and potentially allow for targeted prehabilitation and/or individualized treatment regimens. Using a large multicentre cohort, this study aimed to calculate the incidence of all PD complications and identify risk factors., Method: Data were extracted from the Recurrence After Whipple's (RAW) study, a retrospective cohort study of PD outcomes (29 centres from 8 countries, 2012-2015). The incidence and severity of all complications was recorded and potential risk factors for morbidity, major morbidity (Clavien-Dindo grade > IIIa), postoperative pancreatic fistula (POPF), post-pancreatectomy haemorrhage (PPH) and 90-day mortality were investigated., Results: Among the 1348 included patients, overall morbidity, major morbidity, POPF, PPH and perioperative death affected 53 per cent (n = 720), 17 per cent (n = 228), 8 per cent (n = 108), 6 per cent (n = 84) and 4 per cent (n = 53), respectively. Following multivariable tests, a high BMI (P = 0.007), an ASA grade > II (P < 0.0001) and a classic Whipple approach (P = 0.005) were all associated with increased overall morbidity. In addition, ASA grade > II patients were at increased risk of major morbidity (P < 0.0001), and a raised BMI correlated with a greater risk of POPF (P = 0.001)., Conclusion: In this multicentre study of PD outcomes, an ASA grade > II was a risk factor for major morbidity and a high BMI was a risk factor for POPF. Patients who are preoperatively identified to be high risk may benefit from targeted prehabilitation or individualized treatment regimens., (© The Author(s) 2023. Published by Oxford University Press on behalf of BJS Society Ltd.)

Introduction: Adjuvant chemotherapy (AC) can prolong overall survival (OS) after pancreatoduodenectomy (PD) for pancreatic ductal adenocarcinoma (PDAC). However, fitness for AC may be influenced by postoperative recovery. We aimed to investigate if serious (Clavien-Dindo grade ≥ IIIa) postoperative complications affected AC rates, disease recurrence and OS., Materials and Methods: Data were extracted from the Recurrence After Whipple's (RAW) study (n = 1484), a retrospective study of PD outcomes (29 centres from eight countries). Patients who died within 90-days of PD were excluded. The Kaplan-Meier method was used to compare OS in those receiving or not receiving AC, and those with and without serious postoperative complications. The groups were then compared using univariable and multivariable tests., Results: Patients who commenced AC (vs no AC) had improved OS (median difference: (MD): 201 days), as did those who completed their planned course of AC (MD: 291 days, p < 0.0001). Those who commenced AC were younger (mean difference: 2.7 years, p = 0.0002), more often (preoperative) American Society of Anesthesiologists (ASA) grade I-II (74% vs 63%, p = 0.004) and had less often experienced a serious postoperative complication (10% vs 18%, p = 0.002). Patients who developed a serious postoperative complication were less often ASA grade I-II (52% vs 73%, p = 0.0004) and less often commenced AC (58% vs 74%, p = 0.002)., Conclusion: In our multicentre study of PD outcomes, PDAC patients who received AC had improved OS, and those who experienced a serious postoperative complication commenced AC less frequently. Selected high-risk patients may benefit from targeted preoperative optimisation and/or neoadjuvant chemotherapy., Competing Interests: Declaration of competing interest All authors declare that there are no conflicts of interest., (Copyright © 2023. Published by Elsevier Ltd.)

Appendiceal tumours encompass a wide spectrum of differential diagnoses and frequently present with clinical features of appendicitis. We report the case of a 43-year-old woman who presented with epigastric pain, dyspepsia and bloating. An atypical right para-iliac mass was detected on abdominal ultrasound, and computed tomography (CT) identified an appendiceal tumour. The tumour subtype remained indeterminate following Gallium-68 Dotatate positron emission tomography (PET); however, an appendiceal neuroendocrine tumour was suspected. Surgical resection with laparoscopic en bloc appendicectomy and limited caecectomy was performed, and histopathological assessment confirmed an appendiceal schwannoma. The report is followed by a review of the literature. To our knowledge, there have been fourteen reported cases of appendiceal schwannoma. The preoperative diagnosis can be challenging and appendiceal schwannoma had not been suspected in any of the reported cases, while a suspected diagnosis of neuroendocrine tumour or gastrointestinal stromal tumour was common. Definitive diagnosis requires immunohistochemical assessment and S100 is the hallmark. No personal or family history of underlying neurofibromatosis (NF) type 1 or type 2 has been reported to date. As for other gastrointestinal schwannomas, complete surgical resection is the recommended treatment for appendiceal schwannoma. Following this, despite lack of long-term follow-up, no cases of recurrence have been reported thus far., (© 2022. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Background: Pancreatoduodenectomy (PD) is recommended in fit patients with a resectable ampullary adenocarcinoma (AA). We aimed to identify predictors of five-year recurrence/survival., Methods: Data were extracted from the Recurrence After Whipple's (RAW) study, a multicentre retrospective study of PD patients with a confirmed head of pancreas or periampullary malignancy (June 1st, 2012-May 31st, 2015). Patients with AA who developed recurrence/died within five-years were compared to those who did not., Results: 394 patients were included and actual five-year survival was 54%. Recurrence affected 45% and the median time-to-recurrence was 14 months. Local only, local and distant, and distant only recurrence affected 34, 41 and 94 patients, respectively (site unknown: 7). Among those with recurrence, the most common sites were the liver (32%), local lymph nodes (14%) and lung/pleura (13%). Following multivariable tests, number of resected nodes, histological T stage > II, lymphatic invasion, perineural invasion (PNI), peripancreatic fat invasion (PPFI) and ≥1 positive resection margin correlated with increased recurrence and reduced survival. Furthermore, ≥1 positive margin, PPFI and PNI were all associated with reduced time-to-recurrence., Conclusions: This multicentre retrospective study of PD outcomes identified numerous histopathological predictors of AA recurrence. Patients with these high-risk features might benefit from adjuvant therapy., (Copyright © 2023 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Cholangiocarcinoma (CCA) is an aggressive malignancy arising from the biliary epithelium. It may occur at any location along the biliary tree with the perihilar area being the most common. Prognosis is poor with 5-year overall survival at less than 10%, typically due to unresectable disease at presentation. Radical surgical resection with clear margins offers a chance of cure in patients with resectable tumours, but is frequently not possible due to locally advanced disease. On the other hand, orthotopic liver transplantation (LT) allows for a radical and potentially curative resection for these patients, but has been historically controversial due to the limited supply of donor grafts and previously poor outcomes. In patients with perihilar CCA, within specific criteria and following the implementation of a protocol combining neoadjuvant chemoradiation and LT, excellent results have been achieved in the last decades, resulting in its increasing acceptance as an indication for LT and the standard of care in several centres with significant experience. However, in intrahepatic CCA, the role of LT remains controversial and owing to dismal previous results it is not an accepted indication. Nevertheless, more recent studies have demonstrated favourable results with LT in early intrahepatic CCA, indicating that, under defined criteria, its role may increase in the future. This review highlights the history and contemporary advances of LT in CCA, with particular focus on the improving outcomes of LT in intrahepatic and perihilar CCA and future perspectives., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: The coronavirus disease 2019 (COVID-19) pandemic caused unprecedented disruption to global healthcare delivery. In England, the majority of elective surgery was postponed or cancelled to increase intensive care capacity. Our unit instituted the 'RM Partners Cancer Hub' at the Royal Marsden Hospital in London, to deliver ongoing cancer surgery in a 'COVID-lite' setting. This article describes the operational set-up and outcomes for upper gastrointestinal (UGI) cancer resections performed during this period., Methods: From April 2020 to April 2021, the Royal Marsden Hospital formed the RM Partners Cancer Hub. This approach was designed to coordinate resources and provide as much oncological treatment as feasible for patients across the RM Partners West London Cancer Alliance. A UGI surgical case prioritisation strategy, along with strict infection control pathways and pre-operative screening protocols, was adopted., Results: A total of 231 patients underwent surgery for confirmed or suspected UGI cancer during the RM Partners Cancer Hub, with 213 completed resections and combined 90-day mortality rate of 3.5%. Good short-term survival outcomes were demonstrated with 2-year disease free survival (DFS) and overall survival (OS) for oesophageal (70.8% and 72.9%), gastric (66.7% and 83.3%) and pancreatic cancer resections (68.0% and 88.0%). One patient who developed perioperative COVID-19 during the RM Partners Cancer Hub operation made a full recovery with no lasting clinical sequelae., Conclusion: Our experience demonstrates that the RM Partners Cancer Hub approach is a safe strategy for continuing upper gastrointestinal (GI) resectional surgery during future periods of healthcare service disruption., (© 2022. Crown.)

The management of hepatocellular carcinoma (HCC) remains complex and will continue to rely on the multidisciplinary input of hepatologists, surgeons, radiologists, oncologists and radiotherapists. With the appropriate staging of patients and selection of suitable treatments, the outcomes for HCC are improving. Surgical treatments encompassing both liver resection and orthotopic liver transplantation (OLT) are the definitive curative-intent options. However, patient suitability, as well as organ availability, pose essential limitations. Consequently, non-surgical options, such as ablative techniques, play an increasingly important role, especially in small HCCs, where overall and disease-free survival can be comparable to surgical resection. Ablative techniques are globally recommended in recognised classification systems, showing increasingly promising results. Recent technical refinements, as well as the emerging use of robotic assistance, may expand the treatment paradigm to achieve improved oncological results. At present, in very early stage and early stage unresectable disease, percutaneous thermal ablation is considered the treatment of choice. Owing to their different features, various ablative techniques, including radiofrequency ablation, microwave ablation, cryotherapy ablation and irreversible electroporation, have been shown to confer different comparative advantages and applicability. We herein review the role of available ablative techniques in the current complex multidisciplinary management of HCC, with a main focus on the indications and outcomes, and discuss future perspectives.

Background: Extrapelvic manifestations of endometriosis can be identified in nearly every part of the female body, and the true prevalence of extrapelvic locations is unknown. Pancreatic endometriosis may manifest in several ways, ranging from emergency presentations to asymptomatic cysts., Method: A systematic PubMed and Scopus search was conducted., Results: Eighteen patients from 17 case reports were included. The patients' mean age was 39.3 (range: 21-72) years. An emergency presentation was noted in 8 of the 18 (44.4%) patients. Menstrual irregularity was present in 3 (16.7%) patients, while in 3 (16.7%) cases there was simultaneous presence of endometriosis elsewhere. The most frequent symptoms at presentation of pancreatic endometrial cysts were epigastric pain, acute left upper quadrant pain, back pain, nausea/vomiting/diarrhea, which occurred in 12 (66.7%), 11 (61.1%), 4 (22.2%), and 6 (33.3%) patients, respectively. Only one case presented as an asymptomatic pancreatic cyst. The maximum diameter of the endometrial cysts ranged from 1-16 cm. In the majority of cases, surgical treatment was offered (16/18, 88.9%). Recurrence of pancreatic endometrial cyst occurred in one case only, following needle aspiration of the endometrial cyst. No fatality was reported., Conclusions: Review of the available published literature suggests that pancreatic endometriosis is a rare condition that should be included in the differential diagnosis of pancreatic masses. Further clinical and experimental studies are necessary to investigate the pathogenesis of extrapelvic and pancreatic endometriosis., Competing Interests: Conflict of Interest: None, (Copyright: © Hellenic Society of Gastroenterology.)

Background: Patients with borderline-resectable pancreatic ductal adenocarcinoma (BR-PDAC) have historically poor survival, even after curative pancreatic resection and adjuvant chemotherapy. Emerging evidence suggests that neoadjuvant chemoradiation (NCR) improves R0 resection rates in BR-PDAC patients. We evaluated the R0 resection rate, disease-free survival (DFS) and overall survival (OS) in our patients who underwent NCR for BR-PDAC at our institution., Methods: All patients who underwent NCR for BR-PDAC from January 2010 to March 2020 were included in the study. The patients received a variety of NCR regimens during the study period, and in patients with radiological evidence of tumour stability or regression, pancreatic resection was performed. The primary endpoint was the OS, and the secondary endpoints included patient morbidity, the R0 resection rate, histological parameters and the DFS., Results: The study included 29 patients (16 men and 13 women), with a median age of 65 years (range 46-74 years). Of these 29 patients, 17 received FOLFIRINOX and 12 received gemcitabine (GEM)-based NCR regimens. All patients received chemoradiation at the end of chemotherapy (range 45-56 Gy). R0 resection was achieved in 75% of the patients, with a higher rate noted in the FOLFIRINOX group. The median DFS was 22 months for the whole cohort but higher in the FOLFIRINOX group (34 months). The median OS for the cohort was 29 months, with a higher median OS noted for the FOLFIRINOX cohort versus the GEM cohort (42 versus 28 months)., Conclusion: NCR, particularly FOLFIRINOX-based treatment, for BR-PDAC results in higher rates of R0 resection and an increased median DFS and OS, supporting its continued use in this patient group.

Intrahepatic cholangiocarcinoma (iCCA) is an aggressive malignancy with an increasing incidence worldwide and poor prognosis, despite several advances and continuous efforts to develop effective treatments. Complete surgical resection is the mainstay of treatment and offers a potentially curative option, but is only possible in less than a third of patients, owing to advanced disease. Chemotherapy is a well-established treatment in the adjuvant and palliative setting, however, confers limited benefit. Conventional radiotherapy is challenging due to local toxicity. With recent advances in stereotactic ablative radiotherapy (SABR), it is now possible to focus ablative beams of radiotherapy precisely aimed at tumours to minimise damage to surrounding viscera. This review details the history, technical background and application of SABR to iCCA, with directions for future research suggested., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

On a global scale, colorectal cancer (CRC) is currently the fourth most commonly diagnosed cancer and despite progress in early diagnosis and treatment has the third highest mortality. Patients with oligometastatic disease to the liver may be suitable for liver resection with a curative intent. A sustained progress in perioperative management and surgical techniques, including staged liver resections, has increased the number of patients who may be offered hepatectomy. It is well recognised that early detection of any tumour, including recurrence, leads to a timely initiation of treatment with improved outcomes. Tumour biomarkers have long been desired in the search for a tool to aid cancer diagnosis, prognosis and follow-up. Currently, the only widely used biomarker for CRC, Carcinoembryonic Antigen (CEA), has multiple limitations, clearly illustrating the need for novel biomarkers. It is therefore unsurprising that much research has focused on identifying such markers with the literature being swamped with new and promising biomarkers. The aim of this study is to review the current status and role of circulating biomarkers in patients post hepatectomy for colorectal cancer metastasis including alternative cancer antigens to CEA, extracellular vesicles, circulating microRNA, circulating tumour cells and circulating tumour DNA., Competing Interests: The authors declare no conflict of interest., (© 2022 The Author(s). Published by IMR Press.)

Tumours of the ampulla of Vater are relatively uncommon lesions of the digestive system. They are typically diagnosed at an earlier stage than other types of tumours in this region, due to their tendency to invoke symptoms by obstructing the bile duct or pancreatic duct. Consequently, many are potentially curable by excision. Surgical ampullectomy (SA) (or transduodenal ampullectomy) for an ampullary tumour was first described in 1899, but was soon surpassed by pancreatoduodenectomy (PD), which offered a more extensive resection resulting in a lower risk of recurrence. Ongoing innovation in endoscopic techniques over recent decades has led to the popularization of endoscopic papillectomy (EP), particularly for adenomas and even early cancers. The vast majority of resectable ampullary tumours are now treated using either PD or EP. However, SA continues to play a role in specific circumstances. Many authors have suggested specific indications for SA based on their own data, practices, or interpretations of the literature. However, certain issues have attracted controversy, such as its use for early ampullary cancers. Consequently, there has been a lack of clarity regarding indications for SA, and no evidence-based consensus guidelines have been produced. All studies reporting SA have employed observational designs, and have been heterogeneous in their methodologies. Accordingly, characteristics of patients and their tumours have differed substantially across treatment groups. Therefore, meaningful comparisons of clinical outcomes between SA, PD and EP have been elusive. Nevertheless, it appears that suitably selected cases of ampullary tumours subjected to SA may benefit from favourable peri-operative and long-term outcomes with very low mortality and significantly long survival, hence its role in this setting warrants further clarification, while it can also be useful in the management of specific benign entities. Whilst the commissioning of a randomised controlled trial seems unlikely, well-designed observational studies incorporating adjustments for confounding variables may become the best available comparative evidence for SA, potentially informing the eventual development of consensus guidelines. In this comprehensive review, we explore the role of SA in the modern management of ampullary lesions., Competing Interests: Conflict-of-interest statement: The authors declare no conflict of interests for this article., (©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.)

Primary sclerosing cholangitis (PSC) is a premalignant condition and a well-documented risk factor for cholangiocarcinoma (CCA) which is the most common malignancy in this setting and the leading cause of deaths in the recent years, with an increasing incidence. PSC-associated CCA has a geographical distribution that follows the incidence of PSC, with an observed ascending gradient from the Eastern to the Western and from the Southern to the Northern countries. It may arise at any location along the biliary tree but is most common in the perihilar area. Patients with PSC and intrahepatic or perihilar CCA are typically not suitable for liver resection, which is otherwise the treatment of choice with curative intent in patients with resectable tumours, providing a radical resection with clear margins can be achieved. This largely relates to the commonly advanced stage of liver disease at presentation, which allows consideration for liver resection only for a very limited number of suitable patients with PSC. On the other hand, remarkable progress has been reached in the last decades with the implementation of a protocol combining neoadjuvant chemoradiation and orthotopic liver transplantation (OLT) for the treatment of perihilar CCA, within specific criteria. Excellent results have been achieved particularly for PSC patients with this cancer, who seem to benefit the most from this treatment, having converted this into an accepted indication for transplantation and the standard of care in several experienced centres. Intrahepatic CCA as an indication for OLT remains controversial and has not been accepted given disappointing previous results. However, as recent studies have shown favourable outcomes in early intrahepatic CCA, it may be that under defined criteria, OLT may play a more prominent role in the future. Distal CCA in the context of PSC requires aggressive surgical treatment with curative intent, when feasible. This review provides insight about particular features of CCA in the setting of PSC, with a main focus on its incidence, considerations relating to its anatomical location and implications to treatment and outcomes, through the viewpoint of historical evolution of management, and future perspectives., Competing Interests: Conflict-of-interest statement: The authors declare no conflict of interests for this article., (©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: Elderly ovarian cancer (OC) patients are more likely to be managed suboptimally, with worse clinical outcomes as a result. Strategies to decrease morbidity are lacking. Methodology: Consecutive patients with advanced stage OC (IIIC-IV) who were managed in our center between January 2016 and July 2018 were retrospectively analyzed. All patients underwent neoadjuvant chemotherapy (NACT) and interval debulking surgery (IDS) according to our institution protocol. We divided patients into two groups: Group 1 (age ≥ 70 years) and Group 2 (age < 70 years). The primary outcome of the study was assessment of peri-operative morbidity amongst two groups., Results: A total of 153 patients were referred during the study period. 114 patients underwent IDS after NACT (74.5%), 46 in Group 1 and 68 in Group 2. Elderly patients were more likely to receive more than three cycles of NACT prior to IDS compared to younger patients (39% vs. 19%, p  = 0.03). Elderly patients were more frequently subjected to Cardiopulmonary Exercise Testing (CPET) as pre-operative assessment (63% vs. 27%, p  = 0.002). Optimal/complete resection was achieved in all patients in Group 1 (100%) and in 97% of patients in Group 2. With the exception of higher postoperative cardiac arrhythmias in Group 1 (11% vs. 1%, p  = 0.04), no significant differences in 30-day morbidity were observed. No 90-day death in both groups was registered., Conclusion: Older age should not preclude clinicians from offering ultra-radical resection to patients with advanced OC after NACT. In our series, elderly patients received the same treatment with similar outcomes to the younger group. Clinicians should be encouraged to use CPET for patients' selection following NACT.

Background: Several studies have demonstrated the feasibility and role of bulky cardiophrenic lymph nodes (CPLNs) resection during primary debulking surgery (PDS) for stage IV ovarian cancer (OC). However, no studies, to date, investigated the accuracy and feasibility of CPLNs assessment and removal during interval debulking surgery (IDS) after neoadjuvant chemotherapy (NACT)., Material and Methods: A retrospective analysis of consecutive stage IV OC patients who underwent NACT followed by IDS with CPLNs assessment and/or resection from July 2017 to June 2018. Bulky CPLNs were considered for excision when a full-thickness diaphragmatic resection was required in order to achieve complete tumour resection., Results: A total of 21 ovarian cancer stage IV patients treated with NACT followed by IDS were identified. Seven (33.3%) patients underwent CPLNs resection due to bulky appearance of the CPLNs at the intraoperative palpation. The final histological examination of the CPLNs reported metastatic disease in four (57%) of seven patients. Complete cytoreduction without residual disease was achieved in five cases (71.4%) while in two case (28.6%) optimal cytoreduction was performed. Intra-operative surgical complications occurred in one patient. One patient had a major postoperative complication (Clavien-Dindo 3). Two cases of postoperative cardiac arrhythmia were observed., Conclusions: CPLNs intraoperative assessment is less accurate during IDS compared to previous PDS studies. CPLNs removal during IDS after NACT for stage IV OC could be safely performed to achieve a complete resection.

Objective: The aim of the study was to define histopathologic characteristics that independently predict overall survival (OS) and disease-free survival (DFS), in patients who underwent resection of an ampullary adenocarcinoma with curative intent., Summary Background Data: A broad range of survival rates have been described for adenocarcinoma of the ampulla of Vater, presumably due to morphological heterogeneity which is a result of the different epitheliums ampullary adenocarcinoma can arise from (intestinal or pancreaticobiliary). Large series with homogenous patient selection are scarce., Methods: A retrospective multicenter cohort analysis of patients who underwent pancreatoduodenectomy for ampullary adenocarcinoma in 9 European tertiary referral centers between February 2006 and December 2017 was performed. Collected data included demographics, histopathologic details, survival, and recurrence. OS and DFS analyses were performed using Kaplan-Meier curves and Cox proportional hazard models., Results: Overall, 887 patients were included, with a mean age of 66 ± 10 years. The median OS was 64 months with 1-, 3-, 5-, and 10-year OS rates of 89%, 63%, 52%, and 37%, respectively. Histopathologic subtype, differentiation grade, lymphovascular invasion, perineural invasion, T-stage, N-stage, resection margin, and adjuvant chemotherapy were correlated with OS and DFS. N-stage (HR = 3.30 [2.09-5.21]), perineural invasion (HR = 1.50 [1.01-2.23]), and adjuvant chemotherapy (HR = 0.69 [0.48-0.97]) were independent predictors of OS in multivariable analysis, whereas DFS was only adversely predicted by N-stage (HR = 2.65 [1.65-4.27])., Conclusions: Independent predictors of OS in resected ampullary cancer were N-stage, perineural invasion, and adjuvant chemotherapy. N-stage was the only predictor of DFS. These findings improve predicting survival and recurrence after resection of ampullary adenocarcinoma.

Introduction: Ampullary adenocarcinoma (AAC) is a rare malignancy with great morphological heterogeneity, which complicates the prediction of survival and, therefore, clinical decision-making. The aim of this study was to develop and externally validate a prediction model for survival after resection of AAC., Materials and Methods: An international multicenter cohort study was conducted, including patients who underwent pancreatoduodenectomy for AAC (2006-2017) from 27 centers in 10 countries spanning three continents. A derivation and validation cohort were separately collected. Predictors were selected from the derivation cohort using a LASSO Cox proportional hazards model. A nomogram was created based on shrunk coefficients. Model performance was assessed in the derivation cohort and subsequently in the validation cohort, by calibration plots and Uno's C-statistic. Four risk groups were created based on quartiles of the nomogram score., Results: Overall, 1007 patients were available for development of the model. Predictors in the final Cox model included age, resection margin, tumor differentiation, pathological T stage and N stage (8th AJCC edition). Internal cross-validation demonstrated a C-statistic of 0.75 (95% CI 0.73-0.77). External validation in a cohort of 462 patients demonstrated a C-statistic of 0.77 (95% CI 0.73-0.81). A nomogram for the prediction of 3- and 5-year survival was created. The four risk groups showed significantly different 5-year survival rates (81%, 57%, 22% and 14%, p < 0.001). Only in the very-high risk group was adjuvant chemotherapy associated with an improved overall survival., Conclusion: A prediction model for survival after curative resection of AAC was developed and externally validated. The model is easily available online via www.pancreascalculator.com., Competing Interests: Declaration of competing interest The authors of the abovementioned manuscript have no conflicts of interest to declare., (Copyright © 2020. Published by Elsevier Ltd.)

Background: The benefits of laparoscopic hemi-hepatectomy compared to open hemi-hepatectomy are not clear., Objective: This study aims to share our experience with the laparoscopic hemi-hepatectomy compared to an open approach., Methods: A total of 40 consecutive laparoscopically started hemi-hepatectomy (intention-to-treat analysis) cases between August 2012 and October 2015 were matched against open cases using the following criteria: laterality of surgery and pathology (essential criteria); American Society of Anesthesiologists score, body mass index, pre-operative bilirubin, neo-adjuvant chemotherapy, additional procedures, portal vein embolization, and presence of cirrhosis/fibrosis on histology (secondary criteria); age and gender (tertiary criteria). Hand-assisted and extended hemi-hepatectomy cases were excluded from the study. The two groups were compared for blood loss, operative time, hospital stay, morbidity, mortality, and oncological outcomes. All complications were quantified using the Clavien-Dindo classification., Results: Two groups were well matched (p = 1.00). In the two groups, 10 patients had left and 30 had right hemi-hepatectomy. Overall conversion rate was 15%. Median length of hospital and high dependency unit stay was less in the intention to treat laparoscopic hemi-hepatectomy group: 6 versus 8 days, p = 0.025 and 1 versus 2 days, p = 0.07. Median operative time was longer in the intention to treat laparoscopic hemi-hepatectomy group: 420 min (range: 389.5-480) versus 305 min (range: 238.8-348.8; p = 0.001). Intra-operative blood loss was equivalent, but the overall blood transfusions were higher in the intention to treat laparoscopic hemi-hepatectomy (50 vs 29 units, p = 0.36). The overall morbidity (18 vs 20 patients, p = 0.65), mortality (2.5%), and the positive resection margin status were similar (18% vs 21%, p = 0.76). The 1- (87.5% vs 92.5%, p = 0.71) and 3-year survival (70% vs 72.5%, p = 1.00) was also similar., Conclusions: We observed lower hospital and high dependency unit stay in the laparoscopic group. However, the laparoscopic approach was associated with longer operating time and a non-significant increase in blood transfusion requirements. There was no difference in morbidity, mortality, re-admission rate, and oncological outcomes.

Background: Whether patients who undergo resection of ampullary adenocarcinoma have a survival benefit from adjuvant chemotherapy is currently unknown. The aim of this study was to compare survival between patients with and without adjuvant chemotherapy after resection of ampullary adenocarcinoma in a propensity score-matched analysis., Methods: An international multicentre cohort study was conducted, including patients who underwent pancreatoduodenectomy for ampullary adenocarcinoma between 2006 and 2017, in 13 centres in six countries. Propensity scores were used to match patients who received adjuvant chemotherapy with those who did not, in the entire cohort and in two subgroups (pancreatobiliary/mixed and intestinal subtypes). Survival was assessed using the Kaplan-Meier method and Cox regression analyses., Results: Overall, 1163 patients underwent pancreatoduodenectomy for ampullary adenocarcinoma. After excluding 187 patients, median survival in the remaining 976 patients was 67 (95 per cent c.i. 56 to 78) months. A total of 520 patients (53·3 per cent) received adjuvant chemotherapy. In a propensity score-matched cohort (194 patients in each group), survival was better among patients who received adjuvant chemotherapy than in those who did not (median survival not reached versus 60 months respectively; P = 0·051). A survival benefit was seen in patients with the pancreatobiliary/mixed subtype; median survival was not reached in patients receiving adjuvant chemotherapy and 32 months in the group without chemotherapy (P = 0·020). Patients with the intestinal subtype did not show any survival benefit from adjuvant chemotherapy., Conclusion: Patients with resected ampullary adenocarcinoma may benefit from gemcitabine-based adjuvant chemotherapy, but this effect may be reserved for those with the pancreatobiliary and/or mixed subtype., (© 2020 BJS Society Ltd Published by John Wiley & Sons Ltd.)

Oesophageal sarcomas are very rare while various histological types have occasionally been reported. Malignant Peripheral Nerve Sheath Tumour (MPNST) of the oesophagus is an exceedingly rare type of oesophageal sarcoma with only thirteen cases previously reported in the world literature. However, it should be included in the differential diagnosis of oesophageal neoplasias. Due to the small number of reported cases, the information about the biological behaviour of this entity is still insufficient. While MPNST is generally considered an aggressive type of tumour with high recurrence rates after surgical treatment and poor prognosis, previous reports of cases with oesophageal localization have recorded satisfactory outcomes overall even with less aggressive therapeutic approaches, although a long-term follow-up is lacking. Herein, we present the case of a 76-year-old female patient with oesophageal MPNST who presented with lymph node and distant metastases at the time of diagnosis, accounting for the second time only that this unusual presentation of this extremely uncommon disease has been reported. In our case, the course of disease was extremely aggressive which resulted in the second recorded death from this entity in the literature. The case presentation is followed by an extensive review of the world literature for the so far reported cases, aiming to highlight all relevant aspects such as demographics, clinical features, diagnostic assessment and findings, histological parameters, treatment and prognosis, and extract valuable previously unpublished conclusions for this rare entity., Key Words: Lymph node metastasis, Malignant Peripheral Nerve Sheath Tumour, Malignant schwannoma, Neurogenic sarcoma, Oesophagus, S100.

We present a case report of a patient with Bouveret syndrome with interesting radiological findings and successful surgical treatment after failure of the endoscopic techniques. The report is followed by a review of the literature regarding the diagnostic means and proper treatment of this rare entity. Bouveret syndrome refers to the condition of gastric outlet obstruction caused by the impaction of a large gallstone into the duodenum after passage through a cholecystoduodenal fistula. Many endoscopic and surgical techniques have been described in the management of this syndrome. This is a case of a 78-year-old patient with severe medical history who presented in bad general condition with an 8-day history of nausea, multiple bilious vomiting episodes, anorexia, discomfort in the right hypochondrium and epigastrium, and fever up to 38,5°C. The diagnosis of Bouveret syndrome was set after performing the proper imaging studies. An initial endoscopic effort to resolve the obstruction was performed without success. Surgical treatment managed to extract the impacted gallstone through an enterotomy after removal into the first part of the jejunum.