Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature.
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.


METHODS
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.


CONCLUSIONS
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.
Tsai
Susan
S
Department of Surgery, The Johns Hopkins University School of Medicine, 600 N Wolfe Street, Baltimore, MD 21287, USA.
Gurakar
Ahmet
A
Anders
Robert
R
Lam-Himlin
Dora
D
Boitnott
John
J
Pawlik
Timothy M
TM
D000328:Adult; D016738:Alagille Syndrome / Q000453:epidemiology*; D006528:Carcinoma, Hepatocellular / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D015897:Comorbidity; D005260:Female; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed