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Liver Tumors

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Pediatric Surgery

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Abstract

Liver tumors in pediatric patients present a unique clinical challenge. They are relatively rare, with an incidence of 0.7 per million population. However, two-thirds of primary liver tumors are malignant. They commonly present with nonspecific symptoms until presenting definitively at an advanced stage, with a mass in the right-upper quadrant or epigastrium. Some infantile tumors lack invasive potential, yet they remain life-threatening due to a diffuse nature that may cause high-output cardiac failure, compression of adjacent structures, or risk of malignant degeneration. A normally functioning liver is critical for childhood development, and some tumors are particularly aggressive, disrupting normal physiology and growth. The incidence of liver tumors in Western countries has increased, partially as a result of improved detection via noninvasive imaging. Several tumor types can arise in the setting of viral hepatitis or metabolic liver disease; however, many arise from normal liver parenchyma without any known environmental or genetic risk factors.

The two most common hepatic malignancies in pediatric and adolescent patients are hepatoblastoma and hepatocellular carcinoma. Hepatoblastoma arises in infants and young children, while hepatocellular carcinoma arises in older children. Advances in chemotherapy have greatly improved the outcomes of hepatoblastoma; however, these gains have not proven effective in hepatocellular carcinoma. The most common benign liver tumors are hemangioendothelioma, mesenchymal hamartoma, and focal nodular hyperplasia. Serial imaging, embolization, chemotherapy, surgical resection, and transplantation have been used in the treatment of benign and malignant tumors of the liver. Most data have consistently shown improved overall survival with complete resection, aided by early detection, neoadjuvant therapy, and improved perioperative care. Further improvements will come from multi-institutional and multinational cooperative studies. With the low incidence of these tumors, cooperative groups are vital to collect samples for biologic and genomic analysis, in order to determine exposure risks, prognostic factors, and outcomes data.

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Authors and Affiliations

  1. Department of Surgery, Pediatric Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    David G. Darcy, Joshua N. Honeyman & Michael P. La Quaglia

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  1. David G. Darcy
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  2. Joshua N. Honeyman
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  3. Michael P. La Quaglia
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Correspondence to David G. Darcy .

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Editors and Affiliations

  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

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Darcy, D.G., Honeyman, J.N., La Quaglia, M.P. (2020). Liver Tumors. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_148-1

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  • DOI: https://doi.org/10.1007/978-3-642-38482-0_148-1

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