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Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature

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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.

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Authors and Affiliations

  1. Department of Surgery, The Johns Hopkins University School of Medicine, Harvey 611, 600 N Wolfe Street, Baltimore, MD, 21287, USA

    Susan Tsai & Timothy M. Pawlik

  2. Department of Gastroenterology and Hepatology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Ahmet Gurakar

  3. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Robert Anders, Dora Lam-Himlin & John Boitnott

Authors
  1. Susan Tsai
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  2. Ahmet Gurakar
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  3. Robert Anders
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  4. Dora Lam-Himlin
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  5. John Boitnott
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  6. Timothy M. Pawlik
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Correspondence to Timothy M. Pawlik.

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Tsai, S., Gurakar, A., Anders, R. et al. Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature. Dig Dis Sci 55, 3052–3058 (2010). https://doi.org/10.1007/s10620-009-1123-7

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  • DOI: https://doi.org/10.1007/s10620-009-1123-7

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