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Hepatoblastoma and prune belly syndrome: a potential association

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Abstract

Prune belly syndrome (PBS) is a congenital anomaly characterized by the clinical triad of lax abdominal musculature, bilateral cryptorchidism, and abnormalities of the kidney and urinary tract. Previous reports of malignancy in patients with PBS have been limited to germ cell tumors. Hepatoblastoma (HBL) is the most common hepatic malignancy of childhood, affecting approximately 100 children each year in the USA. We describe a set of 4 pediatric patients with PBS and HBL. All individuals were born after 2002. These subjects lacked genetic, natal, or environmental factors known to confer risk of HBL. The occurrence of PBS and HBL in these patients constitutes a novel potential association.

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Acknowledgements

We wish to thank the patients, families, and healthcare providers for their participation. We thank Dr. Cynthia Pan and Ms. Theresa Kump (Medical College of Wisconsin) for data acquisition. This work was supported by the American Academy of Pediatrics Resident Research Grant to B.B.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Ohio State University College of Medicine, Columbus, OH, 43205, USA

    Brian Becknell, Grace Onimoe, Andrew L. Schwaderer, Mark A. Ranalli & David S. Hains

  2. Division of Nephrology, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, 53201, USA

    Priya Pais

  3. Division of Hematology and Oncology, Nationwide Children’s Hospital, Columbus, OH, 43205, USA

    Grace Onimoe & Mark A. Ranalli

  4. Division of Hematology and Oncology, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, 53201, USA

    Hemalatha Rangarajan

  5. Division of Nephrology, Nationwide Children’s Hospital, Columbus, OH, 43205, USA

    Andrew L. Schwaderer & David S. Hains

  6. Center for Molecular and Human Genetics, Research Institute at Nationwide Children’s Hospital, Columbus, OH, 43205, USA

    Kirk McHugh

  7. Center for Clinical and Translational Research, Columbus, OH, 43205, USA

    Brian Becknell, Priya Pais, Grace Onimoe, Hemalatha Rangarajan, Andrew L. Schwaderer, Kirk McHugh & Mark A. Ranalli

  8. Center for Clinical and Translational Research, Research Institute at Nationwide Children’s Hospital, Columbus, OH, 43205, USA

    David S. Hains

Authors
  1. Brian Becknell
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  2. Priya Pais
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  3. Grace Onimoe
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  7. Mark A. Ranalli
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  8. David S. Hains
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Corresponding author

Correspondence to Brian Becknell.

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Table S1

Established and suspected risk factors for HBL in patients with PBS and HBL. All patients lacked established risk factors for HBL. This was also true with regard to suspected risk factors, which were negative (−) with the two exceptions shown (+). Maternal pre-pregnancy BMI was unknown (U) in two cases (DOC 28 kb)

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Becknell, B., Pais, P., Onimoe, G. et al. Hepatoblastoma and prune belly syndrome: a potential association. Pediatr Nephrol 26, 1269–1273 (2011). https://doi.org/10.1007/s00467-011-1874-1

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  • DOI: https://doi.org/10.1007/s00467-011-1874-1

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