P4720 - Case Report: A Rare Form of Hepatocellular Carcinoma in a Patient With Inflammatory Bowel Disease

Mounica Konjeti, MD, Jessica Musto, MD, MS
University of Wisconsin Hospitals and Clinics, Madison, WI

Introduction: Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC) that typically affects people in their late adolescence or young adulthood without underlying cirrhosis. The association between hepatocellular carcinoma (HCC) and inflammatory bowel disease (IBD) has not been clearly established, but four cases of FLC have been reported, all in patients with ulcerative colitis (UC). FLC tumors are characterized by their unique histology and genomic alteration involving a fusion gene between DNAJB1 and PRKACA. If FLC is unable to be resected (20-25% of cases), clear guidelines for best form of therapy are not established, and the five-year survival rate is 0-5% with a medial survival of 12 months or less. Given the rarity of FLC as well as limited case reports of the association with IBD, we present the case of Ms. A.

Case Description/Methods: Ms. A is a 23-year-old female with no significant past medical history who presented with characteristic symptoms of IBD, confirmed to be Crohn’s via biopsy. She was incidentally found to have a large lobulated exophytic 9 cm hepatic mass with imaging characteristics concerning for HCC. She had surgical resection of liver segments 4/5 (original stage IIIb (pT4)) with negative margins. Tissue pathology revealed fibrolamellar variant of HCC with focal visceral peritoneal involvement and positive small vessel vascular invasion. Months later she developed night sweats and was found to have recurrent FLC involving her portal vein on surveillance MRI. A year after her initial resection, she had a staging laparotomy, portal lymphadenectomy, and resection of the portal mass. Recurrent disease was identified again a few months later with an enlarging portocaval lymph node, requiring right-sided portal lymphadenectomy. She was started on Lenvatinib to decrease risk of recurrence, and fortunately, she has remained in remission since.

Discussion: FLC is a rare primary hepatocellular carcinoma variant, usually originating in non-cirrhotic livers. While curative resection is possible, 50-100% of patients experience relapse and recurrence. There are limited case reports of HCC in IBD patients, and all existing reports of FLC are in patients with UC. Herein we describe a case of FLC in a patient with newly diagnosed Crohn’s disease.


Disclosures:


Mounica Konjeti, MD, Jessica Musto, MD, MS. P4720 - Case Report: A Rare Form of Hepatocellular Carcinoma in a Patient With Inflammatory Bowel Disease, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.