Surveillance recommendations for children with overgrowth syndromes and predisposition to wilms tumors and hepatoblastoma

Jennifer M. Kalish, Leslie Doros, Lee J. Helman, Raoul C. Hennekam, Roland P. Kuiper, Saskia M. Maas, Eamonn R. Maher, Kim E. Nichols, Sharon E. Plon, Christopher C. Porter, Surya Rednam, Kris Ann P. Schultz, Lisa J. States, Gail E. Tomlinson, Kristin Zelley, Todd E. Druley

Research output: Contribution to journalArticle

Abstract

A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America. Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. For HB, we recommend screening with full abdominal ultrasound and alpha-fetoprotein serum measurements every 3 months from birth (or the time of diagnosis) through the fourth birthday. We recommend that when possible, these patients be evaluated and monitored by cancer predisposition specialists. At this time, these recommendations are not based on the differential risk between different genetic or epigenetic causes for each syndrome, which some European centers have implemented. This differentiated approach largely represents distinct practice environments between the United States and Europe, and these guidelines are designed to be a broad framework within which physicians and families can work together to implement specific screening. Further study is expected to lead to modifications of these recommendations.

Original languageEnglish (US)
Pages (from-to)e115-e122
JournalClinical Cancer Research
Volume23
Issue number13
DOIs
StatePublished - Jul 1 2017

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Fingerprint Dive into the research topics of 'Surveillance recommendations for children with overgrowth syndromes and predisposition to wilms tumors and hepatoblastoma'. Together they form a unique fingerprint.

  • Cite this

    Kalish, J. M., Doros, L., Helman, L. J., Hennekam, R. C., Kuiper, R. P., Maas, S. M., Maher, E. R., Nichols, K. E., Plon, S. E., Porter, C. C., Rednam, S., Schultz, K. A. P., States, L. J., Tomlinson, G. E., Zelley, K., & Druley, T. E. (2017). Surveillance recommendations for children with overgrowth syndromes and predisposition to wilms tumors and hepatoblastoma. Clinical Cancer Research, 23(13), e115-e122. https://doi.org/10.1158/1078-0432.CCR-17-0710