Rahul Patel, DO1, Hiral Amin, DO1, Marisa Pope, DO1, Charles Snyder, MD2 1Jefferson Health, Washington Township, NJ; 2Virtua Health System, Camden, NJ
Introduction: Cholangiocarcinoma is a rare biliary tract cancer with a poor prognosis as most patients remain asymptomatic until late stages. Although surgical resection of early-stage tumors are curative, 90% of patients have unresectable carcinoma. We present a case of a woman presenting with obstructive jaundice who was found to have synchronous gallbladder carcinoma, cholangiocarcinoma and hepatocellular carcinoma (HCC).
Case Description/Methods: A 77-year-old female with a history of hypertension, type 2 diabetes and chronic kidney disease arrived to the hospital after experiencing 2 weeks of unintentional weight loss, painless jaundice, pruritus and acholic stools. Family history was pertinent for her son who died of colon cancer. She arrived hemodynamically stable but her lab work was pertinent for hemoglobin of 7.2 g/dL with a baseline of 10-11g/dL, total bilirubin 10.1 mg/dL, direct bilirubin 6.8 mg/dL, alkaline phosphatase 374 U/L. Her hepatic panel reflected an obstructive pattern which was reflected on a magnetic resonance cholangiopancreatography (MRCP) that suspected acute cholecystitis with evidence of perforation and abscess with asymmetric intrahepatic duct dilation. Given the suspicion for an abscess, she underwent a percutaneous cholecystectomy and gallbladder fluid cytology results were suspicious for malignancy. She then underwent endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) with a stricture seen in the upper third of the main bile duct with sphincterotomy and stent placed with no improvement in hepatic function. The ERCP brush biopsy demonstrated adenocarcinoma with positive CK7, S100p, PAX-8 and CDX2, and negative for CK20, TTF-1, Napsin-A, and Arginase. Liver biopsy was positive for adenocarcinoma. Her course was complicated by a right hepatic artery bleed with unsuccessful embolization. She then went into shock and multi-organ failure leading to her demise.
Discussion: This case was rare as we simultaneously diagnosed our patient with cholangiocarcinoma, gallbladder carcinoma and hepatocellular carcinoma during one stay. She had PAX-8 positivity which is not traditionally seen in pancreatobiliary tumors. PAX-8 is a transcription factor for organogenesis of the thyroid gland, kidney and Müllerian system. It is typically expressed in a high percentage of kidney and ovarian cancers. Interestingly our patient did not have evidence of malignancy of renal/uterus/ovaries evidenced by the CT abdomen/pelvis with intravenous contrast.
