Synchronous double primary squamous cell carcinoma and adenocarcinoma of the extrahepatic bile duct: a case report

Abstract

IntroductionSynchronous double cancers of the bile duct are exceptionally rare. We here report a case of synchronous squamous cell carcinoma and adenocarcinoma of the extrahepatic bile duct.Case presentationA 67-year-old Asian man visited our clinic complaining of jaundice and dark urine. Direct hyperbilirubinemia and an elevated cancer antigen 19–9 level were detected. Preoperative abdominal computed tomography and positron emission tomography showed two masses at the bifurcation of the common hepatic duct and at the distal common bile duct. After biliary drainage, we performed radical pylorus-preserving pancreaticoduodenectomy, without resection margin involvement. Pathological findings revealed that the proximal lesion was a squamous cell carcinoma and that the distal lesion was an adenocarcinoma. Both cholangiocarcinomas were confined to the fibromuscular layer, and there was no communication between the two tumors. Multiple conglomerated metastatic tumors were detected in his liver 3 months after surgery. He died 8 months after diagnosis.ConclusionsThe disease displayed very aggressive behavior and a very poor prognosis. The only chance for long-term survival is treatment with radical resection. Preoperative positron emission tomography-computed tomography is useful in detecting occult cancer.

Synchronous double primary cancers of the extrahepatic bile duct: A case report and literature review

“…Here, the 2 cancers were found to be separate entities with different histopathological diagnoses. In the case reported by Yoo et al, it was clear that there were 2 primary cancers because 1 was squamous cell carcinoma and the other was adenocarcinoma [5] . In our case, we diagnosed primary double cancers from histological findings: (1) there was no communication between the 2 cancers, (2) the histological findings of the 2 were different from each other; the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma, and the distal bile duct tumor was a poorly differentiated adenocarcinoma.…”

“…Cases with PBM were excluded. As a result, 4 cases of synchronous double cancers of the extrahepatic bile duct without PBM were identified in the English literature [2] , [3] , [4] , [5] . The findings of these cases are listed in Table 1 , along with those of our case.…”

“… Authors [ref.] Year Patient age (years) Sex Tumor location Histology Clinical symptoms Prognosis Ogawa [2] 2001 69 Male 1.Middle 1.Poor Abdominal distension unkonwn 2.Distal 2.Moderate Bedoui [3] 2011 67 Female 1.Middle 1.None Abdominal pain and jaundice unkonwn 2.Distal 2.None Sumiyoshi [4] 2012 78 Male 1.CHD 1.None Abdominal pain and jaundice 31 months 2.Distal 2.None Yoo [5] 2015 67 Male 1.CHD 1.Squamous Jaundice 8 moths 2.Distal …”

“…Most reports are of synchronous gall bladder and bile duct cancers, and cases are often associated with pancreaticobiliary maljunction (PBM) [1] . Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and to date only 4 cases have been reported in the English literature [2] , [3] , [4] , [5] . We herein report a case of synchronous double primary cancers of the extrahepatic bile duct and review the relevant literature.…”

How to apply clinical cases and medical literature in the framework of a modified “failure mode and effects analysis” as a clinical reasoning tool – an illustration using the human biliary system

“…Liver function test, ERCP, CT or MRI [6]Choledochal cyst may be associated with biliary atresia [59]Malignancy e.g. SCC at the bifurcation of the common hepatic duct, and adenocarcinoma in the common bile duct [60]Biliary obstruction leading to hyperbilirubinemia [60]Jaundice, dark urine, itch, and weight loss [60]Abdominal ultrasound, CT, positron emission tomography-CT [60]Other cases of malignancy at the common bile duct [61, 62]Bile synthesis, conjugation, transportHepatic glucuronidating activity is reduced [63]Mild, chronic unconjugated hyperbilirubinemia in the absence of hepatocellular disease or hemolysis (Gilbert syndrome) [63]Intermittent mild jaundice [64]Genetic testing [64]Reduced expression of bilirubin UDP-glucuronosyltransferase 1 gene; an autosomal recessive mode of inheritance was suggested [63]Deficiency of hepatic glucuronyl transferase [65]Hyperbilirubinemia of the unconjugated type; complication includes kernicterus (Crigler–Najjar syndrome) [65]Jaundice since birth; hepatomegaly; frequent generalized, tonic and clonic convulsions due to kernicterus [65]Serum bilirubin (direct/conjugated and indirect/unconjugated) test, genetic testing [65]This disease is inherited as an autosomal recessive trait [65]A defect in the canalicular multispecific organic anion transporter ( cMOAT ) gene ( ABCC2/MRP2 superfamily) located at 10q24 [66]Impaired hepatobiliary transport of non-bile salt organic anions leading to chronic conjugated hyperbilirubinemia (Dubin–Johnson syndrome) [66]This patient presented with repeated episodes of jaundice during illness; other patients may present with abdominal pain, fatigue, liver enlargement, or dark urine [67] [68]Liver function test, abdominal ultrasound, liver biopsy showed presence of parenchymal pigmentation, urinary coproporphyrin level, genetic testing [67]This is an autosomal recessive disorder [67]Homozygous inactivation of two adjacent genes SLCO1B1 and SLCO1B3 encoding organic anion transporting polypeptides OATP1B1 and OATP1B3 [69]Chronic conjugated hyperbilirubinemia without abnormal hepatic pigmentation (Rotor syndrome) [69, 70]. Abnormal transfer of sulfobromophthalein from plasma into the liver [70]Nonhemolytic jaundice [71]Urinary coproporphyrin and plasma sulfobromoph...…”