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Atypical presentation of an advanced obstructive biliary cancer without jaundice.

Salvador VB, Samrao P, Leytin A, Basith M - Am J Case Rep (2013)

Bottom Line: Unusual natural history/clinical course.The mere presence of Courvoisier's sign, even in the absence of other signs of biliary obstruction, could be suggestive of advanced neoplastic process along the biliary tract.Laboratory evidence of cholestasis might lag behind the clinical severity of the biliary obstruction in cholangiocarcinoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Jamaica, New York, U.S.A.

ABSTRACT

Patient: Female, 60 FINAL DIAGNOSIS: Cholangiocarcinoma Symptoms: Abdominal pain • abdominal discomfort

Medication: - Clinical Procedure: - Specialty: Oncology.

Objective: Unusual natural history/clinical course.

Background: Cholangiocarcinoma remains to be a challenging case to diagnose and manage as it usually presents in advanced stage and survival rate remains dismal despite the medical breakthroughs. It is usually classified as intrahepatic, perihilar or distal tumor which can lead to bile duct obstruction causing sluggish flow of bile through the biliary tract and promoting increased absorption of bilirubin, bile acids and bile salts into systemic circulation accounting for the occurrence of jaundice, dark-colored urine and generalized pruritus. It usually becomes symptomatic when the tumor has significantly obstructed the biliary drainage causing painless jaundice and deranged liver function with cholestatic pattern. Jaundice occurs in 90% of the cases when the tumor has obstructed the biliary drainage system. A markedly dilated gallbladder as initial presenting feature in the absence of other typical obstructive clinical manifestations of an advanced stage of the cholangiocarcinoma is rare.

Case report: This case report presents an atypical case of an elderly woman who presented with advanced metastatic ductal cholangiocarcinoma with markedly dilated gallbladder and liver mass without other clinical manifestations and laboratory evidence of cholestatic jaundice.

Conclusions: The mere presence of Courvoisier's sign, even in the absence of other signs of biliary obstruction, could be suggestive of advanced neoplastic process along the biliary tract. Laboratory evidence of cholestasis might lag behind the clinical severity of the biliary obstruction in cholangiocarcinoma.

No MeSH data available.


Related in: MedlinePlus

MRCP of the biliary tree showing the narrowing on the mid common bile duct (arrow).
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f3-amjcaserep-14-462: MRCP of the biliary tree showing the narrowing on the mid common bile duct (arrow).

Mentions: A 60-year-old female comes in with 3-day duration of worsening right upper quadrant and lower abdominal pain described as sharp, constant, non-radiating without associated fever, chills, jaundice, weight loss, nausea, vomiting or changes in bowel movement. Her past medical comorbidities were hypertension, hyperlipidemia and osteoporosis. Physical exam revealed anicteric sclerae, soft abdomen with normal bowel sounds, palpable gallbladder and left lobe of the liver, no abdominal tenderness or guarding, no splenomegaly appreciated. Pelvic exam was remarkable for palpable right-sided pelvic mass. EGD was normal. Colonoscopy showed non-bleeding diverticulosis. MRI with contrast revealed a hypointense left hepatic lobe mass measuring 5.3 cm (craniocaudad) × 4.2 cm (AP) × 5.3 cm (transverse) with heterogenous and hyperintense T2 signal on T2 weighted sequences; an enlarged gallbladder (12.2×6×6.4 cm) with cholelithiasis without pericholecystic fluid (Figures 1 and 2). MRCP revealed intrahepatic biliary ductal dilation measuring approximately 1 cm with abrupt short segment narrowing in the extrahepatic common bile duct measuring 0.2 cm in diameter ( Figure 3). The distal common bile duct measures normal in diameter. There was no obvious filling defect to suggest choledocholithiasis. Initial hepatic function panel was deranged showing combined mild cholestatic pattern without bilirubinemia [Alkaline phosphatase: 89 U/L (30–115 U/L), AST: 32 U/L (5–40 U/L), ALT: 30 U/L (5–50 U/L), GGT: 91 U/L (3–40 U/L), LDH: 262 U/L (90–225 U/L), albumin: 3.5 g/dL (3.5–5 g/dL), total bilirubin: 0.52 mg/dL (0–1.5 mg/dL), bilirubin conjugated: 0.13 mg/dL (0–0.3 mg/dL)]. Tumors markers were significant for markedly elevated CA19-9 (12394 U/mL) with moderate elevation in CEA (394 ng/mL) and CA-125 (85 U/mL) while alpha fetoprotein tumor marker was within normal limits (3.89 ng/mL). Hepatitis A, B and C panels were nonreactive. ANA was negative while anti-mitochondrial antibody and anti-smooth muscle antibody were both positive at low titers, 1:20 and 1:40, respectively. Her coagulation profile was also within normal limits [PTT: 30 seconds (26–34 seconds), PT: 11.7 seconds (10–12.5 seconds) and INR 1.03]. CT-guided liver biopsy was obtained and histopathological exam revealed high-grade carcinoma ( Figure 4). Epithelial nature was confirmed by positive stains for cytokeratins ( Figure 5). Patient was then referred to Surgical Oncology Service.


Atypical presentation of an advanced obstructive biliary cancer without jaundice.

Salvador VB, Samrao P, Leytin A, Basith M - Am J Case Rep (2013)

MRCP of the biliary tree showing the narrowing on the mid common bile duct (arrow).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3823417&req=5

f3-amjcaserep-14-462: MRCP of the biliary tree showing the narrowing on the mid common bile duct (arrow).
Mentions: A 60-year-old female comes in with 3-day duration of worsening right upper quadrant and lower abdominal pain described as sharp, constant, non-radiating without associated fever, chills, jaundice, weight loss, nausea, vomiting or changes in bowel movement. Her past medical comorbidities were hypertension, hyperlipidemia and osteoporosis. Physical exam revealed anicteric sclerae, soft abdomen with normal bowel sounds, palpable gallbladder and left lobe of the liver, no abdominal tenderness or guarding, no splenomegaly appreciated. Pelvic exam was remarkable for palpable right-sided pelvic mass. EGD was normal. Colonoscopy showed non-bleeding diverticulosis. MRI with contrast revealed a hypointense left hepatic lobe mass measuring 5.3 cm (craniocaudad) × 4.2 cm (AP) × 5.3 cm (transverse) with heterogenous and hyperintense T2 signal on T2 weighted sequences; an enlarged gallbladder (12.2×6×6.4 cm) with cholelithiasis without pericholecystic fluid (Figures 1 and 2). MRCP revealed intrahepatic biliary ductal dilation measuring approximately 1 cm with abrupt short segment narrowing in the extrahepatic common bile duct measuring 0.2 cm in diameter ( Figure 3). The distal common bile duct measures normal in diameter. There was no obvious filling defect to suggest choledocholithiasis. Initial hepatic function panel was deranged showing combined mild cholestatic pattern without bilirubinemia [Alkaline phosphatase: 89 U/L (30–115 U/L), AST: 32 U/L (5–40 U/L), ALT: 30 U/L (5–50 U/L), GGT: 91 U/L (3–40 U/L), LDH: 262 U/L (90–225 U/L), albumin: 3.5 g/dL (3.5–5 g/dL), total bilirubin: 0.52 mg/dL (0–1.5 mg/dL), bilirubin conjugated: 0.13 mg/dL (0–0.3 mg/dL)]. Tumors markers were significant for markedly elevated CA19-9 (12394 U/mL) with moderate elevation in CEA (394 ng/mL) and CA-125 (85 U/mL) while alpha fetoprotein tumor marker was within normal limits (3.89 ng/mL). Hepatitis A, B and C panels were nonreactive. ANA was negative while anti-mitochondrial antibody and anti-smooth muscle antibody were both positive at low titers, 1:20 and 1:40, respectively. Her coagulation profile was also within normal limits [PTT: 30 seconds (26–34 seconds), PT: 11.7 seconds (10–12.5 seconds) and INR 1.03]. CT-guided liver biopsy was obtained and histopathological exam revealed high-grade carcinoma ( Figure 4). Epithelial nature was confirmed by positive stains for cytokeratins ( Figure 5). Patient was then referred to Surgical Oncology Service.

Bottom Line: Unusual natural history/clinical course.The mere presence of Courvoisier's sign, even in the absence of other signs of biliary obstruction, could be suggestive of advanced neoplastic process along the biliary tract.Laboratory evidence of cholestasis might lag behind the clinical severity of the biliary obstruction in cholangiocarcinoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Jamaica, New York, U.S.A.

ABSTRACT

Patient: Female, 60 FINAL DIAGNOSIS: Cholangiocarcinoma Symptoms: Abdominal pain • abdominal discomfort

Medication: - Clinical Procedure: - Specialty: Oncology.

Objective: Unusual natural history/clinical course.

Background: Cholangiocarcinoma remains to be a challenging case to diagnose and manage as it usually presents in advanced stage and survival rate remains dismal despite the medical breakthroughs. It is usually classified as intrahepatic, perihilar or distal tumor which can lead to bile duct obstruction causing sluggish flow of bile through the biliary tract and promoting increased absorption of bilirubin, bile acids and bile salts into systemic circulation accounting for the occurrence of jaundice, dark-colored urine and generalized pruritus. It usually becomes symptomatic when the tumor has significantly obstructed the biliary drainage causing painless jaundice and deranged liver function with cholestatic pattern. Jaundice occurs in 90% of the cases when the tumor has obstructed the biliary drainage system. A markedly dilated gallbladder as initial presenting feature in the absence of other typical obstructive clinical manifestations of an advanced stage of the cholangiocarcinoma is rare.

Case report: This case report presents an atypical case of an elderly woman who presented with advanced metastatic ductal cholangiocarcinoma with markedly dilated gallbladder and liver mass without other clinical manifestations and laboratory evidence of cholestatic jaundice.

Conclusions: The mere presence of Courvoisier's sign, even in the absence of other signs of biliary obstruction, could be suggestive of advanced neoplastic process along the biliary tract. Laboratory evidence of cholestasis might lag behind the clinical severity of the biliary obstruction in cholangiocarcinoma.

No MeSH data available.


Related in: MedlinePlus