Distal cholangiocarcinoma (dCCA) is a rare malignancy arising from the epithelial cells of the distal biliary tract and has a poor prognosis. dCCA is often clinically silent and patients commonly present with locally advanced and/or distant disease. For patients identified with early stage, resectable disease, surgical resection with negative margins remains the only curative treatment strategy available. However, despite appropriate treatment and diligent surveillance, risk of recurrence remains high with nearly 50% of patients experiencing recurrence at 5 years subsequent to surgical resection; therefore, it is prudent to continue to optimize neoadjuvant and adjuvant therapies in order to reduce the risk of recurrence and improve overall survival. In this review, we discuss the clinical presentation, workup and surgical treatment of dCCA.

/pdf/surgical-treatment-of-distal-cholangiocarcinoma-1o6rc5s0.pdf

Surgical Treatment of Distal Cholangiocarcinoma

Biliary stenosis may represent a diagnostic and therapeutic challenge resulting in a delay in diagnosis and initiation of therapy due to the frequent difficulty in distinguishing a benign from a malignant stricture. In such cases, the diagnostic flowchart includes the sequential execution of imaging techniques, such as magnetic resonance, magnetic resonance cholangiopancreatography, and endoscopic ultrasound, while endoscopic retrograde cholangiopancreatography is performed to collect tissue for histopathological/cytological diagnosis or to treat the stenosis by insertion of stent. The execution of percutaneous transhepatic drainage with subsequent biopsy has been shown to increase the possibility of tissue diagnosis after failure of the above techniques. Although the diagnostic yield of histopathology and imaging has increased with improvements in endoscopic ultrasound and peroral cholangioscopy, differential diagnosis between malignant and benign stenosis may not be easy in some patients, and strictures are classified as indeterminate. In these cases, a multidisciplinary workup including biochemical marker assays and advanced technologies available may speed up a diagnosis of malignancy or avoid unnecessary surgery in the event of a benign stricture. Here, we review recent advancements in the diagnosis and management of biliary strictures and describe tips and tricks to increase diagnostic yields in clinical routine.

Tips and tricks for the diagnosis and management of biliary stenosis-state of the art review.

Background: Elevated serum bilirubin has been shown to be a reliable predictor of pancreatic and biliary malignancy but the relationship between serum bilirubin and inadequate (C1), benign (C2) and indeterminate (C3) cellular samples has not been explored. The aim of this study is to determine the relationship between serum bilirubin and pancreatic, biliary or ampullary malignancy in the context of non-confirmatory cytology. Methods: This is a retrospective analysis of patients with obstructive jaundice undergoing investigation for possible pancreatic, peri-ampullary or biliary malignancy between 2009 and 2013. Results: 135 patients were included; 84 had a malignant diagnosis and 51 benign. All patients with C4 or C5 cytology (n=49) had confirmed malignancy. 35 out of 86 C1 – C3 samples were falsely negative. ROC curve analysis demonstrated a strong association (AUC 0.912) between elevated serum bilirubin and malignancy; serum bilirubin ≥ 100 µmol/L had a sensitivity of 86% and a specificity of 88%. In the C1-C3 subgroup, this association was maintained (AUC 0.905). Serum bilirubin ≥ 100 µmol/L had a sensitivity of 80% and specificity of 88%. Using this cut-off highlighted 28 out of 35 of the malignancies missed by cytology (p = 0.003). Conclusion: Our study demonstrates that a serum bilirubin ≥ 100 µmol/L is associated with malignancy and this relationship is maintained in C1-C3 cytology. When faced with non-confirmatory cytology in the absence of a benign aetiology and an elevated serum bilirubin ≥ 100 µmol/L, we advocate more aggressive investigation to avoid missing an occult malignancy.

/pdf/serum-bilirubin-levels-can-predict-pancreatic-and-biliary-23ml93949w.pdf

Serum Bilirubin Levels Can Predict Pancreatic and Biliary Malignancies in Patients with Obstructive Jaundice and Non-conclusive Cytology

Double duct sign (DDS) (dilated common bile and pancreatic duct) is synonymous with pancreatic head/peri-ampullary tumor (PHPAT). There is limited evidence on whether incidental DDS (I-DDS) is associated with an increased risk of malignancy. This study aimed to evaluate 5-year outcomes of I-DDS.Patients were categorized according to their risk of malignancy. 'Low-risk' patients, including those with I-DDS between 2010 and 2015, were analyzed in this study. The primary outcome was incidence of PHPAT within five years of identification of DDS. Histology results from endoscopic ultrasound-guided biopsy were considered diagnostic. Secondary outcomes were incidence of benign causes, extent of follow-up investigations, and clinical indicators of malignancy in patients with DDS.Among 103 patients with DDS, 20 had I-DDS. Subsequent follow-up of these 20 patients found no patient with PHPAT, two (10%) patients with chronic pancreatitis, and 18 (90%) patients with no cause found. The median follow-up duration for 'low-risk' patients was 7.3 years (range, 6-11 years). The mean number of follow-up investigations per patient was two (range, 0-9). Investigations included computed tomography (n = 27), magnetic resonance cholangiopancreatography (n = 23), endoscopy (n = 16), and ultrasound (n = 14). Patients with jaundice were more likely to have malignancy (p < 0.01). Those with abdominal pain were more likely to have a benign cause (p < 0.01). Hyperbilirubinemia and/or deranged liver enzymes and raised CA19-9 were more likely to be associated with PHPAT (p < 0.01).Patients with I-DDS have a low risk of developing PHPAT within five years. 

Incidental double duct sign: Should we be worried? Results from a long-term follow-up study

/pdf/dajung-geomcul-jeonsanhwadanceungcwalyeong-yeongsangeseo-2py5pyorm1.pdf

다중 검출 전산화단층촬영 영상에서 바터 팽대부의 샘근종과 국소적 샘암종의 감별

Background To date, no studies have sought to determine the frequency of malignancy in patients presenting with a putative biliary stricture and normal liver function tests (LFTs). The primary aim of this retrospective cohort study was to determine the likelihood of malignancy in patients presenting with a biliary stricture and normal LFTs, a normal bilirubin level either alone or in combination with normal levels of liver enzymes [alkaline phosphatase (ALP) and alanine transaminase (ALT)]. A secondary aim was to determine any clinical/biochemical/sonographic features that may be associated with malignancy. Methods Patients presenting over a 10‐year period were included. Fifteen variables were analysed to determine their association with malignant disease. Results Eight hundred and thirty patients with putative biliary strictures were included. Primary hepatopancreaticobiliary (HPB) cancers presented with a normal bilirubin and normal liver enzymes (ALP and ALT) in 6% of cases. Patients with a putative biliary stricture and a normal bilirubin level whose final diagnoses were pancreatic cancer, ampullary cancer, distal cholangiocarcinoma and hilar cholangiocarcinoma represented 21%, 13%, 7% and 9% of individuals diagnosed with these pathologies, respectively. Hypoalbuminaemia and isolated intrahepatic duct dilatation on ultrasound were significantly associated with malignancy in patients with normal bilirubin and completely normal LFTs. Conclusions This study has shown that patients with a putative biliary stricture and completely normal LFTs are unlikely to have a primary HPB malignancy. Those presenting with a normal bilirubin level, but deranged liver enzymes (ALP and/or ALT), are more likely to have malignant disease, and this should necessitate a higher degree of clinical suspicion.

Malignant biliary strictures in patients with a normal bilirubin and/or normal liver enzymes

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Malignant biliary strictures in patients with a normal bilirubin and/or normal liver enzymes