Protocol for the CoNoR Study: A prospective multi-step study of the potential added benefit of two novel assessment tools in colorectal liver metastases technical resectability decision-making.

INTRODUCTION: Liver resection is the only curative treatment for colorectal liver metastases (CLM). Resectability decision-making is therefore a key determinant of outcomes. Wide variation has been demonstrated in resectability decision-making, despite the existence of criteria. This paper summarises a study protocol to evaluate the potential added value of two novel assessment tools in assessing CLM technical resectability: the Hepatica preoperative MR scan (MR-based volumetry, Couinaud segmentation, liver tissue characteristics and operative planning tool) and the LiMAx test (hepatic functional capacity). METHODS AND ANALYSIS: This study uses a systematic multistep approach, whereby three preparatory workstreams aid the design of the final international case-based scenario survey:Workstream 1: systematic literature review of published resectability criteria.Workstream 2: international hepatopancreatobiliary (HPB) interviews.Workstream 3: international HPB questionnaire.Workstream 4: international HPB case-based scenario survey.The primary outcome measures are change in resectability decision-making and change in planned operative strategy, resulting from the novel test results. Secondary outcome measures are variability in CLM resectability decision-making and opinions on the role for novel tools. ETHICS AND DISSEMINATION: The study protocol has been approved by a National Health Service Research Ethics Committee and registered with the Health Research Authority. Dissemination will be via international and national conferences. Manuscripts will be published. REGISTRATION DETAILS: The CoNoR Study is registered with ClinicalTrials.gov (registration number NCT04270851). The systematic review is registered on the PROSPERO database (registration number CRD42019136748).

Fibrolamellar carcinoma: Challenging the challenge.

Fibrolamellar carcinoma (FLC) is a rare and poorly understood malignancy, which seems to be more prevalent in young patients compared with conventional hepatocellular carcinoma (HCC). Performing prospective clinical trials recruiting patients diagnosed with FLC has proven challenging with scarce data available guiding clinical management. The use of a number of chemotherapy compounds in these patients, including cisplatin, epirubicin, 5-fluorouracil (5-FU) and recombinant interferon α-2B (IFN-α-2B), has been reported in the literature, mainly in the form of case reports. The most promising systemic therapy tested so far is the combination of 5-FU infusion and 3-weekly IFN-α-2B, based on results from a phase II clinical trial. This article provides an overview of our own experience with this treatment schedule for patients with FLC, confirming its activity and treatment-derived benefit in the real world. Current challenges being faced by healthcare professionals treating patients with advanced FLC are discussed, especially the increasingly limited access to IFN-α-2B, which could compromise the access to an active therapy in the coming future, and the difficulties in the development of new treatment options for advanced FLC.

Review of the diagnosis, classification and management of autoimmune pancreatitis.

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, with as yet undetermined incidence and prevalence in the general population. Our understanding of it continues to evolve. In the last few years, 2 separate subtypes have been identified: type 1 AIP has been recognised as the pancreatic manifestation of a multiorgan disease, named immunoglobulin G4 (IgG4)-related disease while type 2 AIP is a pancreas specific disorder not associated with IgG4. International criteria for the diagnosis of AIP have been defined: the HISORt criteria from the Mayo clinic, the Japan consensus criteria and, most recently, the international association of pancreatology "International Consensus Diagnostic Criteria". Despite this, in clinical practice it can still be very difficult to confirm the diagnosis and differentiate AIP from a pancreatic cancer. There are no large studies into the long-term prognosis and management of relapses of AIP, and there is even less information at present regarding the Type 2 AIP subtype. Further studies are necessary to clarify the pathogenesis, treatment and long-term outcomes of this disease. Critically for clinicians, making the correct diagnosis and differentiating the disease from pancreatic cancer is of the utmost importance and the greatest challenge.

A space occupying lesion masquerading as pancreatic carcinoma.

The coexistence of painless jaundice and a space-occupying lesion in the head of the pancreas usually signifies a diagnosis of pancreatic cancer. We present a case, where the cause of a pancreatic mass turned out to be related to tuberculosis. Tuberculosis affecting abdominal organs in isolation is uncommon, and more often forms part of disseminated disease. Pancreatic tuberculosis is very rare, especially in immunocompetent individuals. While every effort should be made to ensure that potentially operable pancreatic cancers undergo prompt surgical excision, the challenge for the future will be to make a preoperative diagnosis of pancreatic conditions that require medical rather than surgical therapy.

Radiation exposure in patients with inflammatory bowel disease--primum non nocere?

BACKGROUND: Patients with Inflammatory Bowel disease (IBD) are frequently exposed to diagnostic medical radiation for the diagnosis and evaluation of their disease. Despite increasing awareness of the potentially serious downstream effects, few studies have quantified radiation exposure in IBD patients. METHODS: We conducted a retrospective review of 280 consecutive patients attending IBD clinics at our hospital. All incidences of diagnostic medical radiation from initial diagnosis until 30 June 2010 inclusive were recorded. The radiation dose for each procedure was obtained from standardized tables and the effective dose for each procedure calculated by multiplying this value by the number of procedures during the study period. The sum of doses received was the cumulative effective dose. RESULTS: The mean and median cumulative effective radiation doses were 10.17 mSv and 4.12 mSv respectively. Crohn's disease patients had significantly higher cumulative effective radiation exposure than UC patients (p < 0.001) with exposure exceeding 10 mSv in 58.3%, 25 mSv in 18.1% and 50 mSv in 6.3%, respectively. Smoking status, disease duration, and previous surgery were significant predictors for increased radiation exposure even after adjusting for other predictors. 47 small bowel magnetic resonance (MR) studies were undertaken as an alternative to ionising radiation in the last 3 years. CONCLUSIONS: Patients with IBD, particularly those with Crohn's disease, are exposed to significant amounts of diagnostic medical radiation in their lifetime. Clinicians must remain vigilant to the risk of cumulative radiation when evaluating these patients and consider non-ionizing alternatives such as intestinal ultrasound and MR imaging where clinically appropriate.

Complications arising in simple and polycystic liver cysts.

Liver cysts are common, affecting 5%-10% of the population. Most are asymptomatic, however 5% of patients develop symptoms, sometimes due to complications and will require intervention. There is no consensus on their management because complications are so uncommon. The aim of this study was to perform a collected review of how a series of complications were managed at our institutions. Six different patients presenting with rare complications of liver cysts were obtained from Hepatobiliary Units in the United Kingdom and The Netherlands. History and radiological imaging were obtained from case notes and computerised radiology. As a result, 1 patient admitted with inferior vena cava obstruction was managed by cyst aspiration and lanreotide; 1 patient with common bile duct obstruction was first managed by endoscopic retrograde cholangiopancreatography and stenting, followed by open fenestration; 1 patient with ruptured cysts and significant medical co-morbidities was managed by percutaneous drainage; 1 patient with portal vein occlusion and varices was managed by open liver resection; 1 patient with infected cysts was treated with intravenous antibiotics and is awaiting liver transplantation. The final patient with a simple liver cyst mimicking a hydatid was managed by open liver resection. In conclusion, complications of cystic liver disease are rare, and we have demonstrated in this series that both operative and non-operative strategies have defined roles in management. The mainstays of treatment are either aspiration/sclerotherapy or, alternatively laparoscopic fenestration. Medical management with somatostatin analogues is a potentially new and exciting treatment option but requires further study.

Laparoscopic distal pancreatectomy for a large pancreatic lymphoepithelial cyst: a rare entity.

Lymphoepithelial cysts (LECs) of the pancreas are rare true pancreatic cysts. Their cause is not known. The differential diagnosis is broad and includes many benign and malignant cystic lesions of the pancreas and surrounding organs. A combination of imaging modalities and fine needle aspiration might narrow the differential diagnosis. However, the final diagnosis can only be achieved with certainty after resection of the cyst. In this study, we report the largest LEC of the pancreas to have been resected laparoscopically. A 43-year-old man presented with upper abdominal pain, a 7.5 cm mutlioculated cystic mass in the pancreatic body and tail on imaging, and a raised serum cancer antigen-19-9. Laparoscopic distal pancreatectomy and splenectomy was performed. Histologic examination revealed a LEC. This study discusses the diagnostic difficulties and management decisions which face surgeons treating pancreatic cysts.

Efficacy of renal artery angioplasty and stenting in a solitary functioning kidney.

OBJECTIVE: The aim of this study was to share our experience of percutaneous renal artery angioplasty and stenting (PTRAS) in our patients with a solitary functioning kidney over a 10-year period. METHODS: The procedures were performed on 75 patients from 1995 to 2004. Data were collected retrospectively from case notes of patients. The definition for solitary functioning kidney was a contralateral kidney size of <8 cm, complete occlusion of contralateral renal artery or previous nephrectomy. Serum creatinine was considered improved or worse if the deviation from the baseline value was >20%. RESULTS: For the purpose of halting renal deterioration (n=47), there were improvement and stabilization in 21% and 55% at 3 months and 28% and 28% at 12 months. Systolic blood pressure (n=27) improvement and stabilization were achieved in 33% and 56% both at 3 and 12 months. Diastolic blood pressure (n=27) improvement and stabilization were 22% and 70% at 3 months and 33% and 48% at 12 months. Five out of seven patients with acute renal failure (serum creatinine>500 micromol/l and requiring haemodialysis) pre-procedure were dialysis-free at 12 months. Complications occurred in 19 (25%) patients and these included bleeding (n=16), pseudoaneurysm (n=3), renal artery dissection (n=2) and cholesterol embolization (n=1). CONCLUSION: PTRAS in a solitary functioning kidney produced clinical benefits in the majority of patients with resistant hypertension and renal function deterioration.