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Rare malignant liver tumors in children

Pediatric Radiology volume 49, pages 1404–1421 (2019)Cite this article

Abstract

Malignant hepatic tumors in children are rare, comprising 1.3% of all pediatric malignancies. Following hepatoblastoma, hepatocellular carcinoma is the second most common. Other malignant hepatic tumors seen in childhood include those of mesenchymal origin including undifferentiated embryonal sarcoma, angiosarcoma, rhabdomyosarcoma and epithelioid hemangioendothelioma, as well as biliary tumors such as cholangiocarcinoma. Diagnosis can be challenging because of their rarity, and the recognition of distinctive imaging features for certain tumors such as epithelioid hemangioendothelioma and biliary rhabdomyosarcoma can focus the differential diagnosis and expedite the diagnostic process. A complete MRI examination with hepatocyte-specific contrast media and diffusion-weighted imaging helps to focus the differential diagnosis, and, although findings are often nonspecific, in some cases typical features on MRI can be helpful in diagnosis. Histopathological analysis is usually required for definitive diagnosis. Hepatic tumors tend to be aggressive, and full staging is imperative to establish disease extent. Significant proportions are not amenable to upfront surgical resection and often require a multimodality approach including neoadjuvant chemotherapy within a multidisciplinary setting. Facilitating complete surgical resection is usually required for better survival. In this review, we emphasize pathology and imaging features for rare liver tumors that are useful in reaching a prompt diagnosis. We also discuss general clinical findings, prognosis and management of these tumors.

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Authors and Affiliations

  1. Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G1X8, Canada

    Govind B. Chavhan

  2. Medical Imaging Department, University of Toronto, Toronto, ON, Canada

    Govind B. Chavhan

  3. Department of Pediatric Laboratory Medicine,, The Hospital for Sick Children,, Toronto, ON, Canada

    Iram Siddiqui

  4. Department of Pediatrics, University of Toronto, Toronto, ON, Canada

    Iram Siddiqui, Katrina M. Ingley & Abha A. Gupta

  5. Department of Hematology and Oncology,, The Hospital for Sick Children,, Toronto, Ontario, Canada

    Katrina M. Ingley & Abha A. Gupta

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  1. Govind B. Chavhan
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  2. Iram Siddiqui
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  3. Katrina M. Ingley
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  4. Abha A. Gupta
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Correspondence to Govind B. Chavhan.

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Chavhan, G.B., Siddiqui, I., Ingley, K.M. et al. Rare malignant liver tumors in children. Pediatr Radiol 49, 1404–1421 (2019). https://doi.org/10.1007/s00247-019-04402-8

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