What is the biology of pediatric colorectal tumors?

Updated: Jun 06, 2020
  • Author: Jaime Shalkow, MD, FACS; Chief Editor: Cameron K Tebbi, MD  more...
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Answer

Answer

In patients aged 21 years or younger, high-grade tumors are predominant, with signet-ring cell histology (a feature associated with advanced stage and poorer prognosis) reported in 45% of cases in one cohort. Mucinous histology was reported in 80% of adolescents and young adults and 62% of children with CRC in observational studies. [3]

Microsatellite instability among adolescents and young adults with CRC may be a favorable prognostic indicator.

CRC oncogenesis studies have shown tumors in adolescents and young adults to be more complex than those in patients 45 years and older, and often associated with P53 and PTEN mutations. Interestingly, no PIK3CA mutations have been found in patients younger than 50 years, whereas such mutations are seen in 4-7% of older patients.

Attention is currently directed toward precision medicine. Tumor and genomic mutational analyses are increasingly being used to guide treatment options for patients with malignancies. Nicholson et al studied more than 400 patients with CRC and found that loss of Bcl-2 expression is associated with decreased disease-specific and overall survival. This finding could help identify the subset of patients with a more aggressive phenotype and guide therapy choices. [50]

Particular molecular profiles may identify patients with higher-risk disease, who may benefit from more aggressive treatment or specific targeted therapies.


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