Primary sclerosing cholangitis (PSC) is a recognised risk factor for hilar cholangiocarcinoma (hCCA). In selected patients, neoadjuvant chemoradiotherapy followed by liver transplantation provides the optimal chance of long-term survival. However, for the patient described in the present case report, at the time of the patient s treatment, the UK did not have an approved transplant programme for cholangiocarcinoma, and access to liver transplantation was limited, often necessitating upfront surgical resection despite its complexity and limited curative potential. The present study describes the case of a 52-year-old male patient with PSC who was diagnosed with hCCA and underwent an extended right hepatectomy. After 26 months, progressive liver dysfunction due to PSC-related cirrhosis prompted liver transplantation, which was approved following a lengthy appeals process. Over the following years, the patient developed metastases in the bowel, lungs and abdominal wall, all of which were successfully managed with surgical resections. He remained disease-free for 8 years following his initial diagnosis before developing intrahepatic recurrence. The tumour was HER2-positive, and the compassionate use of zanidatamab was initiated following progression on standard therapies. At the time of the writing of the present case report, the patient remained alive 101 months following this initial diagnosis. On the whole, the present case report highlights the potential impact of tumour biology and multimodal treatment in PSC-associated hCCA. The prolonged survival of the patient despite delayed transplant and metastatic recurrence suggests that PSC-related hCCA may follow a more indolent course compared to de novo cases. Future efforts are required to focus on tumour profiling and stratified therapeutic approaches to better guide treatment in this complex disease.

BACKGROUND:Polycystic liver disease (PLD) comprises a spectrum of inherited disorders characterised by progressive cyst development and highly variable clinical manifestations. A significant subset of patients develops debilitating symptoms and despite advances, a unified treatment algorithm is lacking. AIM:To review current evidence for medical, interventional and surgical management of PLD and identify gaps preventing an integrated, evidence-based care pathway. SUMMARY:Somatostatin analogues (SSAs) are the principal disease-modifying therapy, producing modest but reproducible liver-volume reductions of 3-7% and clinically meaningful symptom improvement in selected patients with diffuse small-to-medium cystic disease. Other pharmacologic strategies have shown limited or inconsistent benefit and currently have no established role outside research settings. Interventional radiologic and surgical options provide more substantial debulking but are phenotype-dependent. Partial hepatectomy offers the largest volume reduction but carries high morbidity and liver transplantation (LT) remains the only curative option for advanced disease, with excellent long-term outcomes but significant perioperative risk. Across all modalities, heterogeneous endpoints limit meaningful comparison and hinder integration of therapies into a unified treatment pathway. CONCLUSION:A phenotype-driven management framework and coordinated research strategy incorporating standardised volumetrics, symptom scoring and prospective multicentre cohorts are urgently needed to define optimal sequencing of medical, interventional and surgical therapies in PLD.

Background: Liver regeneration has been the subject of extensive study for many years in an effort to find ways to manipulate the organ's unique ability to regenerate and, thus, push liver resection boundaries further. Since its inception, portal vein embolization (PVE) has been the standard practice for augmenting the future liver remnant (FLR); however, post-hepatectomy liver failure (PHLF) remains a dreaded clinical entity. Recent application of additional embolization of the hepatic veins appears to achieve superior results in both volume augmentation and functional adequacy of the regenerated liver. Here we present our experience with clinical outcomes of extended right hepatectomy (ERH) after PVE compared to those obtained in the extended liver vein deprivation (eLVD) era. Methods: This retrospective study includes all cases that underwent ERH within King's College Hospital, from September 2016 to March 2022. Indications for surgery were mainly cholangiocarcinoma (CCA) and colorectal liver metastases (CRLM). PVE and eLVD were applied when FLR was assessed as <30%. Volumetric analysis of FLR prior to and after PVE and eLVD, and clinical outcomes were analyzed. Results: From September 2016 to March 2022, 42 patients underwent ERH with volume manipulation procedures in our institution. Of those, 23 underwent PVE and 19 eLVD in order to produce FLR augmentation prior to ERH. The eLVD group showed a statistically significantly higher FLR regeneration with mean value 55.7% when compared to the 46.2% in the PVE group (P=0.03). Major complication rate (Clavien-Dindo >= IIIB) was 26.1% (6/23) in the PVE group versus 10.5% (2/19) in the eLVD group. Rates of PHLF (13.0% vs. 5.3%), 30-day mortality (8.7% vs. 0%), and 90-day mortality (13.0% vs. 5.3%) did not differ significantly between the PVE and eLVD groups (P>0.99). The incidence of postoperative ascites was lower in the eLVD group [36.8% (7/19)] than in the PVE group [65.2% (15/23)], although this difference did not reach statistical significance (P=0.07). The need for surgical portal flow modulation was markedly reduced in the eLVD group (5.2%) compared with the PVE group (69.5%) (P=0.001). Conclusions: PHLF is the most fearful complication; thus, FLR regeneration methods are integral part of the preoperative management. Whether FLR volume increase is in concordance with the functional adequacy of the regenerated part of the liver, is widely debated and a matter of further studies. The risk factors and surrogate markers that affect this balance are yet to be determined. This is the first study comparing clinical outcomes using these two methods to achieve FLR augmentation in a cohort of ERH (segments I and IV-VIII) exclusively. Results suggest functional superiority of the FLR in the eLVD group, demonstrated by less ascites and a significantly reduced need for PFM in this highly selected ERH cohort. Based on current evidence, eLVD should be considered the new standard of care prior to ERH, while further studies are warranted to elucidate the underlying mechanisms of liver regeneration.

Arterioportal fistula (APF) is a rare complication of percutaneous liver biopsy. While most biopsy-induced shunts are asymptomatic and resolve on their own, persistent high-flow lesions can lead to progressive vascular remodeling, creating significant management challenges. A 49-year-old woman presented 17 years after undergoing a percutaneous liver biopsy, complaining of a pulsatile epigastric mass. Computed tomography revealed a large aneurysmal intrahepatic APF involving the middle hepatic artery and left portal vein. Due to the complexity of the anatomy and concerns about portal perfusion, endovascular embolization was not pursued. Instead, the patient underwent a formal left hepatectomy, which effectively controlled the fistulous area. Histopathological examination confirmed chronic arterialization of portal venous structures, accompanied by fibromyxoid intimal thickening, consistent with long-standing high-flow shunting. This case illustrates the delayed progression of biopsy-induced APF and emphasizes that hepatic resection is a definitive and safe option for anatomically complex lesions when embolization is not feasible. Careful preoperative mapping of arterial inflow and portal venous outflow is essential for effective operative planning and safe resection in complex APFs.

Neuroendocrine carcinomas of the ampulla of Vater are rare but highly aggressive, poorly differentiated neoplasms associated with poor outcomes. This contrasts with ampullary adenocarcinoma, which is generally associated with more favourable survival following curative resection, and with well-differentiated periampullary neuroendocrine tumours, which typically demonstrate more indolent behaviour and better prognosis. A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-compliant systematic review was conducted across PubMed/MEDLINE, Embase, Scopus, Web of Science, and Google Scholar (from inception to July 2025) to identify reported cases of large-cell neuroendocrine carcinoma (LCNEC) of the ampulla of Vater. Studies reporting extractable patient-level clinicopathological, treatment, and outcome data were included. Data were standardised and synthesised descriptively due to the rarity and heterogeneity of the condition. A total of 22 patient-level cases of LCNEC of the ampulla of Vater were identified from 14 studies. The median age was 70 years (range: 44-84), with a male predominance. Tumours were generally small at presentation (median size approximately 20 mm); however, lymph node metastasis was present in 16 of 20 evaluable patients (80%). All patients underwent pancreaticoduodenectomy or pylorus-preserving pancreaticoduodenectomy. Adjuvant chemotherapy was administered in 12 of 22 patients (55%), most commonly platinum-based regimens. Tumour recurrence occurred in approximately 55-60% of cases, most frequently involving the liver. Median overall survival was approximately 11 months, with eight patients (36%) alive at the last follow-up. LCNEC of the ampulla of Vater is an aggressive malignancy with poor prognosis despite surgical resection. This review highlights a clinically important "size-biology paradox," whereby relatively small tumours demonstrate early metastatic behaviour and adverse outcomes. Tumour biology appears to outweigh conventional staging parameters, underscoring the need for early recognition, accurate histopathological classification, and consideration of systemic therapy. Further multicentre studies are required to optimise management strategies and improve patient outcomes.

Bile duct tumours are most commonly primary cholangiocarcinomas, and upfront surgical resection is often undertaken with curative intent, as preoperative histological confirmation is not routinely required. Melanoma, an aggressive malignancy of neural crest origin, can metastasize widely, but involvement of the biliary tract is exceedingly rare. Even rarer is primary melanoma of the bile duct. The present report describes a 44-year-old man presenting with obstructive jaundice and a mass in the common bile duct (CBD) presumed to represent cholangiocarcinoma. The patient underwent pancreaticoduodenectomy, but histopathology unexpectedly revealed malignant melanoma. Comprehensive staging and dermatological assessment excluded a cutaneous, mucosal or ocular primary, supporting the diagnosis of primary CBD melanoma. To the best of our knowledge, this represents the 14th reported case. This case highlights that melanoma, although rare, should be considered in the differential diagnosis of biliary obstruction, especially in younger patients, as it mandates a distinct postoperative oncological management pathway.

Locally advanced pancreatic ductal adenocarcinoma (LA-PDAC) is increasingly managed with neoadjuvant chemotherapy with the aim of tumour downstaging and potential conversion to resection. LA-PDAC is typically defined by tumour involvement of major vascular structures precluding upfront surgical resection in the absence of distant metastases. However, only a minority of patients ultimately undergo secondary surgery, and pathological complete response (pCR) remains rare. A 64-year-old male was diagnosed in 2011 with LA-PDAC involving the pancreatic neck with vascular involvement precluding upfront resection. He received systemic chemotherapy with capecitabine-cisplatin, followed by four cycles of FOLFIRINOX (oxaliplatin, irinotecan, leucovorin, and 5-fluorouracil). Restaging imaging demonstrated marked tumour regression without metastatic disease. In December 2012, he underwent total pancreatectomy and splenectomy. Histopathological examination revealed complete tumour regression (ypT0N0R0). The patient remained free of pancreatic cancer recurrence for over 13 years and ultimately died from an unrelated primary lung malignancy. Although pCR following neoadjuvant therapy for PDAC is uncommon and associated with improved survival, long-term disease-free survival beyond a decade after conversion surgery for initially unresectable disease is rarely reported. This case highlights the potential impact of tumour biology, sustained systemic response, and multidisciplinary reassessment in selected patients with LA-PDAC.

Ectopic pancreas, also known as heterotopic pancreas, is a congenital anomaly characterised by pancreatic tissue located outside its normal anatomical location, without structural, vascular, or ductal continuity with the native pancreas. Although often asymptomatic, an ectopic pancreas may present as an abdominal mass and radiologically mimic other pathologies, resulting in diagnostic uncertainty and frequently leading to surgical intervention. We report two cases: a 49-year-old man who presented with abdominal discomfort and fullness and was found to have a 2 cm lesion at the duodenojejunal flexure. Workup failed to delineate the nature of the lesion, and the patient underwent resection, revealing ectopic pancreas. A 33-year-old female presented with symptoms of small bowel obstruction. Extensive workup, including capsule endoscopy, revealed an intraluminal polypoid lesion, and underwent laparotomy and resection. In both cases, histopathology confirmed ectopic pancreas. These cases highlight the diagnostic challenges that ectopic pancreas may pose and emphasise its role as a true "chameleon" of abdominal pathology. Its variable clinical and radiological presentations underscore the importance of considering ectopic pancreas in the differential diagnosis of symptomatic gastrointestinal masses.

Metastatic pancreatic cancer carries a poor prognosis, with palliative chemotherapy offering limited survival benefit and conversion to resectability considered rare. The present study reports the case of a 60-year-old woman with pancreatic adenocarcinoma and extensive liver metastases treated with palliative FOLFIRINOX, achieving complete radiological disappearance of hepatic disease and sustained control of the primary tumour. After a treatment pause and subsequent reinitiation of chemotherapy for local progression, the liver metastases remained absent. At 32 months post-diagnosis, the patient underwent pancreatic resection with negative margins and remains alive and free from hepatic recurrence 84 months from diagnosis. This case highlights that select patients may achieve long-term survival through individualized, multimodal treatment strategies.

Background Biliary cystic neoplasms (BCNs), comprising biliary cystadenomas (BCAs) and cystadenocarcinomas (BCACs), are rare cyst-forming epithelial tumours of the liver and biliary tract. Differentiation between benign and malignant lesions remains challenging preoperatively, and current imaging criteria lack sufficient discriminatory power. This study aimed to correlate preoperative imaging features with final histology to better identify radiologic markers of malignancy in BCN. Methodology A retrospective analysis was conducted on 42 histologically confirmed cases of BCAs or BCACs discussed at a specialist hepatopancreatobiliary multidisciplinary team between 2010 and 2023. All cases had preoperative cross-sectional imaging (CT and/or MRI or magnetic resonance cholangiopancreatography). Imaging studies were reviewed for features including wall enhancement, septations, mural nodules, calcification, and internal fluid complexity. Histology was categorised into low-grade dysplasia (LGD), high-grade dysplasia (HGD), or invasive carcinoma (BCAC). Results Of the 42 cases, 39 (93%) were LGD BCA, two (5%) were HGD BCA, and one (2%) was BCAC. Wall enhancement was significantly more common in advanced histology (67%) compared to LGD (28%), and thick fluid density also correlated with advanced pathology (33% vs. 8%). Mural nodules were present only in LGD cases (10%) and absent in HGD or BCAC. The sensitivity and specificity of wall enhancement for advanced histology were 67% and 72%, respectively. Notably, ovarian-type stroma (OTS), a diagnostic criterion in the WHO 2022 classification, was absent in 24% of cases, yet these were still reported as BCAs based on traditional morphological features. Conclusions Wall enhancement and complex fluid characteristics are the most suggestive imaging features of advanced BCN; however, significant overlap with benign lesions limits their diagnostic accuracy. Mural nodules were not predictive of malignancy in this cohort. Our findings support surgical resection of all complex or atypical cystic liver lesions and highlight the variability in real-world histopathological classification, especially regarding OTS. Multidisciplinary consensus and histologic evaluation remain critical for definitive diagnosis.

Background Liver transplantation using a left lateral segment (LLS) graft is a lifesaving procedure for pediatric recipients. While long-term graft function may remain well preserved, biliary complications can arise over time, threatening both graft viability and patient well-being. In some cases, endoscopic or radiological interventions may be infeasible or fail to provide durable relief. We present a case of a biliary anastomotic stricture following pediatric LLS transplantation, successfully managed using a rarely performed but technically valuable surgical approach, that of intrahepatic hepaticojejunostomy known as Longmire type I procedure. Case Report A 21-year-old female, who underwent LLS liver transplantation at 8 months of age for acute liver failure, presented with recurrent episodes of cholangitis. She had previously undergone complex biliary revision with 2 separate hepaticojejunostomies, but subsequently developed a stricture mainly involving the segment II bilio-enteric anastomosis. Radiological intervention failed to traverse the stricture. A segmental intrahepatic cholangio-jejunostomy, commonly referred to as the Longmire type I procedure, was performed successfully, relieving the obstruction and preserving graft function. Conclusion Biliary complications remain the Achilles’ heel of long-term liver transplant outcomes. While endoscopic and radiological interventions are typically first-line, surgical revision of hepaticojejunostomy is sometimes required. However, re-operative biliary surgery carries significant risks, especially in the context of previous revisions. Intrahepatic cholangio-jejunostomy offers a viable alternative in selected patients, as demonstrated in this case, which is the first of its kind reported in the literature following pediatric living donor liver transplantation.

Hepatic angiosarcoma (HA) is a rare and aggressive malignancy. Radiological findings are non-specific and often mimic benign liver pathologies. Patients' rapid clinical deterioration is often alarming, leading clinicians to a late, futile diagnosis. We present two cases of HA in which presenting symptoms, but more importantly, radiological appearances, were misleading, mimicking liver cysts and cavernous haemangiomas, respectively. Rapid clinical deterioration and speedy radiological evolution of disease spread led to a diagnosis of HA with a dismal prognosis. A 77-year-old male was diagnosed with an incidental finding of a cystic lesion in the upper abdomen. On contrast-enhanced computed tomography (CECT), the lesion mimicked a complex liver cyst with atypical radiological features, prompting clinicians to treat it with surgical resection for an otherwise unclear malignant pathology. On repeat CECT prior to surgery, the disease explosion with extensive peritoneal spread was surprising. A 79-year-old female presented with right flank pain, and CECT showed features of a cavernous haemangioma on the right liver lobe. The MRI confirmed atypical features of an otherwise benign entity, not long before the patient presented with spontaneous rupture, treated with embolisation. On repeat CECT, findings of new multiple liver lesions representing disease spread led to a biopsy confirming HA. Radiological appearances of HA are non-specific and may mimic benign liver pathologies, misleading clinicians. Early radiological detection and clinician awareness may lead to timely diagnosis, as complete resection of this aggressive malignancy offers better outcomes. Recognised treatment options appear limited in most cases, and future molecular analyses of this aggressive cancer may help advance systemic therapies.

Castleman disease (CD) is a group of rare lymphoproliferative disorders characterized by shared histopathological features but distinct clinical entities, broadly classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD involves a single anatomical site and typically follows a benign clinical course, whereas MCD affects multiple lymph node stations and is associated with systemic symptoms and a more complex therapeutic approach. The disease is poorly understood, and the difficulty in reaching a diagnosis is well noted in the literature. While MCD is systemic and requires hematological work-up, abdominal UCD consists of a radiologically detected solitary mass that poses a diagnostic challenge, often necessitating a hepato-pancreatico-biliary (HPB) opinion. We report a retrospective case series of four patients diagnosed with UCD between 2011 and 2022 at a tertiary centre. All patients underwent extensive diagnostic work-up due to suspected malignancy based on radiological features and metabolic imaging. Surgical resection was performed in all cases, given diagnostic ambiguity or concern for malignancy. The cohort included three males and one female, aged 24 to 69 years. Lesions were located in the retroperitoneum, pancreaticoduodenal groove, small bowel mesentery, and adjacent to the caudate lobe. In one patient, UCD coexisted with a head of pancreas adenocarcinoma. In all cases, definitive diagnosis was established following surgical resection and histopathological analysis. One patient was found to have a coexisting focus of follicular dendritic cell sarcoma and remains free of recurrence 12 years post-resection. This rare association has been reported in the context of hyaline-vascular UCD and carries potential malignant behaviour, underscoring the need for long-term surveillance. All patients were referred to hematology services. UCD carries a low malignant potential; however, affected individuals may have an increased risk of developing lymphoproliferative disorders. HPB surgeons should maintain a high index of suspicion for this rare entity when evaluating retroperitoneal, paraduodenal, or mesenteric masses. In the majority of cases, surgical resection represents the culmination of an often complex diagnostic process that poses significant challenges to clinicians and leads patients to undergo surgery in the absence of a definitive preoperative diagnosis. Although complete surgical resection is considered curative and is typically associated with favourable outcomes, the future role of surgery may be subject to re-evaluation, particularly as advancements in radiological modalities could potentially facilitate non-invasive diagnosis. Regardless of the surgical outcome, all patients should be referred to hematology services for long-term follow-up. This case series underscores the diagnostic difficulties posed by UCD and highlights the importance of multidisciplinary collaboration in the management of such cases.

Extended right hepatectomy (ERH) for hilar cholangiocarcinoma (HCCA) is a complex procedure associated with a high risk of postoperative complications. We present a case of ERH complicated by a biliary fistula at the hepatico-jejunostomy site, which led to a severe anastomotic stricture. Traditional percutaneous transhepatic drainage repeatedly failed in this case. The stricture was ultimately treated with trans-jejunal metal stent insertion, successfully restoring bilio-enteric drainage. A 62-year-old male patient was diagnosed with HCCA. After staging, he underwent liver vein deprivation to augment the future liver remnant (FLR), followed by curative resection via ERH (segments I and IV-VIII). Postoperatively, a bile leak at the anastomotic site resulted in a bilio-cutaneous fistula. While conservative management led to a gradual resolution, the fistula caused a tight anastomotic stricture, leading to obstructive jaundice. Traditional percutaneous transhepatic drainage attempts repeatedly failed to traverse the lengthy stricture. Consequently, a novel procedure of inserting a metal biliary stent via a percutaneous puncture of the jejunal loop was attempted. Under CT guidance, the Roux loop was catheterized and distended with contrast, and a guidewire was positioned inside. The patient was then transferred to the angiography suite. Under fluoroscopy, a transjugular intrahepatic portosystemic shunt needle punctured the bile duct stump, providing access to the Roux loop. A fully covered metal stent was successfully deployed crossing the anastomosis re-establishing bilio-enteric drainage. This novel radiological intervention salvaged the remnant liver when standard approaches had failed. Hepatico-jejunostomy stricture following ERH is a critical postoperative complication that can severely compromise FLR function. Surgical options in such cases are limited and pose significant risks. Radiological intervention offers a promising alternative, enabling effective drainage even in the most challenging postoperative scenarios.

Haemorrhagic gallbladder (HG) is a very rare complication of acute cholecystitis. Cases are not frequently described in the literature, but frail elderly patients on anticoagulants are the primary group of patients affected. Semi-elective or emergency laparoscopic cholecystectomy is the treatment of choice, while haemorrhagic shock requiring emergency laparotomy is an extremely rare event. Primary sclerosing cholangitis (PSC) is an autoimmune liver disease (AiLD), and its association with bleeding complications remains a clinical observation that lacks robust scientific evidence and comprehensive understanding. Here, we present a case of gallbladder rupture causing haemorrhagic shock in a young patient with a background of cirrhotic PSC. A 29-year-old female with PSC and a history of splenic artery aneurysm previously treated with embolization presented to the emergency department complaining of abdominal pain. A striking feature in the biochemistry results was the high bilirubin level without clinical detection of jaundice. Computed tomography (CT) showed a ruptured gallbladder with active bleeding and haemoperitoneum. Emergency laparotomy was mandated as the patient was becoming progressively haemodynamically unstable. The necrosed gallbladder was resected, and blood was evacuated from the abdominal cavity. The patient recovered well with no postoperative complications observed. HG causing haemodynamic shock is quite a rare complication of acute cholecystitis. This is the first case reported in the literature of internal haemorrhage due to HG in a patient with a background of PSC. This case alerts clinicians dealing with PSC patients who may be susceptible to rare complications of common pathologies. AiLD and their relationship with bleeding events remains unclear and subject of future studies.