Multidisciplinary management in the treatment of intrahepatic cholangiocarcinoma

A 63‐year‐old woman who was a former smoker with a past medical history of hypertension and gastroesophageal reflux disease initially presented with upper abdominal pain. Her family history was notable for breast cancer in her mother, lung cancer in her father, and renal cell carcinoma in her sister. An ultrasound showed a heterogenous mass in the left lobe of the liver measuring 8.7 � 7.0 � 5.1 cm that was abutting the common bile duct and concerning for a neoplasm (Figure 1A). On laboratory testing, her alpha fetoprotein (AFP) was elevated (15.7 ng/mL), carbohydrate antigen 19‐9 (CA 19‐9) was normal (<15 U/ml), and carcinoembryonic antigen (CEA) was slightly elevated (0.6 ng/ml). She underwent an ultrasound‐guided biopsy that demonstrated cytokeratin 7 (CK7)‐positive, poorly differentiated adenocarcinoma with nonmucinous gland formation and papillary architecture within sclerotic stroma. Given that the biopsy was positive for CK7 with negative hepatocellular (hepatocyte‐specific antigen, arginase, glypican), CDX2, TTF1, and synaptophysin markers, the mass was diagnosed as an intrahepatic cholangiocarcinoma (iCCA). A computed tomography (CT) scan of the chest, abdomen, and pelvis did not show any extrahepatic metastatic disease but did show a central left hepatic lobe mass in segment 4a/4b that measured 7.7 � 6.7 cm with calcifications suggestive of iCCA (Figure 1B,C). A CT scan also revealed potential tumor thrombus within the middle hepatic vein and distal left portal vein branches, extrahepatic (periportal, gastrohepatic, peripancreatic, portacaval) lymphadenopathy, left intrahepatic biliary ductal dilation, and common bile duct dilation. The patient was started on gemcitabine, cisplatin, and nanoparticle albumin‐bound paclitaxel (nab‐paclitaxel). After 3 months of chemotherapy, the patient's AFP increased to 43.8 ng/ml and her CA 19‐9 increased to 21.4 U/ml. On repeat CT scan, the size of the tumor was stable, but there was suspected intraductal extension toward the central inferior aspect of segment 4b. Given her suboptimal response to chemotherapy, radiation oncology was consulted. Approximately 4 months after starting chemotherapy, the patient underwent yttrium‐90 radioembolization (Y90 RE) to the left hepatic hemiliver and subsequently was resumed on a gemcitabine, cisplatin, and nab‐paclitaxel regimen (Figure 2A). A CT scan 5 months after starting treatment and 1 month after Y90 RE demonstrated a stable left hepatic lobe mass with interval necrosis. However, this effect was mostly seen in the tumor in the left lobe of the liver, whereas there was still some residual arterial enhancement along the right side of the mass because where the tumor extended into the right lobe was not treated given concern of toxicity to the remaining liver. After nine cycles of chemotherapy and the Y90 RE treatment, re‐staging CT scans did not demonstrate any metastatic disease, and the tumor in the left lobe of the liver had a seemingly good response to the Y90 RE (Figure 2B). In addition, the periportal, portacaval, and gastrohepatic lymphadenopathy had decreased in size, and there was no new or progressive adenopathy. At this point, the patient was taken to the operating room, and an extended left hepatectomy, cholecystectomy, and extensive lymphadenectomy, including skeletonizing of the hilum, left hepatic artery, bile ducts, and common hepatic artery, was performed. On postoperative day 5, the patient was tachycardic, a CT scan showed a