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TP53

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Children and young people who have the following cancers will be offered referral for genetic testing for TP53. Referral will be made at the child cancer treatment centre which is providing treatment for the child's cancer.

  • Adrenocortical carcinoma
  • Choroid Plexus carcinoma
  • Anaplastic rhabdomyosarcoma
  • Sonic hedgehog medulloblastoma over 2 years old at diagnosis
  • ALL with hypodiploidy
  • Children with cancer who have a family history which is strongly suggestive of Li Fraumeni Syndrome.

If P53 positive, these children will be offered entry to a formal surveillance programme based on that used in Toronto.

At present, there is insufficient evidence of the efficacy of surveillance in the following 2 groups to recommend testing or surveillance:

  • Siblings of children with cancer who are P53 carriers
  • Children of P53 positive parents

References

  1. Andrea Villani, Uri Tabori, Joshua Schiffman etal. Biochemical and imaging surveillance in germline TP53 mutation carriers with Li-Fraumeni syndrome: a prospective observational study. Lancet Oncol 2011;12:559-67
  2. Mandy L Ballinger, Gillian Mitchell, David Thomas. Surveillance recommendations for patients with germline TP53 mutations. Curr Opin Oncol 2015, 27:332-337
  3. Kate A. McBride, Mandy L. Balinger, Emma Killick etal. Li-Fraumeni syndrome:cancer risk assessment and clinical management. Nat. Rev. Clin. Oncol. 11,260-271 (2014)
  4. eviQ cancer treatments online. Risk management for Li-Fraumeni Syndrome in Children. Last modified 29 Oct 2015

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Document Control

  • Date last published: 18 May 2016
  • Document type: Clinical Guideline
  • Services responsible: National Child Cancer Network
  • Owner: Scott Macfarlane
  • Review frequency: 1 year

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