Kelsey Collins, MD1, Ray Dong, MD2, Frederick Rozenshteyn, MD1, Edward Lung, MD3
1Mount Sinai Beth Israel Medical Center / Icahn School of Medicine at Mount Sinai, New York, NY; 2Mount Sinai Beth Israel Medical Center, New York, NY; 3Mount Sinai St. Luke's and Mount Sinai Roosevelt, New York, NY
Introduction: Cholangiocarcioma (CCA), a malignancy that originates from the epithelial cells of the biliary tree, is rare and often portends a poor prognosis with a survival rate of 5-10% at 5 years. CCA is even more infrequent during pregnancy with only a few reported cases.
Case Description/Methods: A 37-year-old, 31-week pregnant female, presented to the ED with worsening epigastric pain for 3 months. She also had jaundice for several days. Her total bilirubin was 6.5, ALP was 631, ALT was 86, and AST was 80. Lipase was normal. CA 19-9 was elevated to 4649. She had an abdominal US that showed gallbladder sludge, 1.0 cm CBD, and coarse hepatic echogenicity. She underwent MRCP, which revealed CBD dilatation to 1.1 cm, leading up to a 1.7 cm segment of obstruction at the distal CBD, where there was an ill-defined heterogeneous soft tissue mass. Distal to the lesion, the CBD was normal in caliber, with slight prominence of the pancreatic duct at the pancreatic body. In addition, there were 2 hepatic lesions. ERCP was then done for further diagnostic workup. The CBD was deeply cannulated and injected with contrast, with brisk flow up to the hepatic ducts. The middle third of the CBD contained a 25 mm area of severe, localized stenosis, and the upper third of the CBD was dilated to 18 mm. Sphincterotomy was done, and a partially covered metal stent was placed in the CBD with good position and good bile flow. She then proceeded to undergo a CT-guided liver biopsy of the largest hepatic lesion, which ultimately showed moderately differentiated adenocarcinoma with extensive tumor necrosis and desmoplasia. Immunohistochemistry was diffuse and strongly positive for cytokeratin 7 and cytokeratin 19. Findings were all consistent with extrahepatic cholangiocarcinoma with intrahepatic metastasis. Oncology was consulted, who recommended starting a course of Abraxane, Gemcitabine, and Cisplatin, which she deferred until after childbirth. She continued to follow up with Oncology outpatient, but ultimately had uncontrollable disease. She passed away in inpatient hospice due to liver failure and polymicrobial bacteremia likely due to bowel leak and pneumobilia.
Discussion: CCA represents 3% of all gastrointestinal malignancies, and is extremely uncommon during pregnancy. Symptoms and labs can be similar to those seen in HELLP, obstructive cholestasis, and acute fatty liver of pregnancy. A full diagnostic workup is fundamental in pregnant patients presenting with abdominal pain, jaundice, and abnormal liver enzymes.
Citation: Kelsey Collins, MD; Ray Dong, MD; Frederick Rozenshteyn, MD; Edward Lung, MD. P0952 - INTRAUTERINE PREGNANCY COMPLICATED BY A DISTAL COMMON BILE DUCT MASS: AN UNLIKELY DIAGNOSIS OF EXTRAHEPATIC CHOLANGIOCARCINOMA WITH INTRAHEPATIC METASTASIS. Program No. P0952. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.