Faculty Collaboration Database

Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature. Dig Dis Sci 2010 Nov;55(11):3052-8

Date

01/29/2010

Pubmed ID

20108035

DOI

10.1007/s10620-009-1123-7

Scopus ID

2-s2.0-78149358943 (requires institutional sign-in at Scopus site)   25 Citations

Abstract

BACKGROUND: Alagille syndrome is a multi-system developmental disorder associated with paucity of interlobular bile ducts and cholestasis, rarely associated with hepatocellular carcinoma. Associated syndromic co-morbidities may complicate surgical management. As such, we herein review the modern management of a large hepatocellular carcinoma in an adult patient with Alagille syndrome and review the literature of adult Alagille patients with hepatocellular carcinoma.

CASE PRESENTATION: A 29-year-old woman with a history of Alagille syndrome was referred with biopsy-proven 12 × 8 cm hepatocellular carcinoma replacing her right liver. Biopsy of the contralateral liver demonstrated findings consistent with Alagille syndrome, but no underlying cirrhosis. CT volumetrics demonstrated a future liver remnant of 40%. Extensive hematologic and cardiac work-up was performed pre-operatively, given the syndrome's associated bleeding dyscrasias and cardiac abnormalities. The patient underwent a margin-negative right hepatectomy using the "hanging" technique through a thoracoabdominal approach. The patient developed a transient hyperbilirubinemia but no hepatic insufficiency and did well post-operatively.

CONCLUSION: Since Alagille syndrome affects multiple organ systems, preoperative evaluation of cardiac, hematologic, and hepatic function should be considered. This case illustrates the peri-operative management of an Alagille patient, and highlights several key technical points that contributed to a successful resection.

Author List

Tsai S, Gurakar A, Anders R, Lam-Himlin D, Boitnott J, Pawlik TM

MESH terms used to index this publication - Major topics in bold

Adult
Alagille Syndrome
Carcinoma, Hepatocellular
Comorbidity
Female
Hepatectomy
Humans
Liver Neoplasms
Magnetic Resonance Imaging
Tomography, X-Ray Computed