Colorectal cancer in the very young: a comparative study of tumor markers, pathology and survival in early onset and adult onset patients

J Pediatr Surg. 2016 Nov;51(11):1812-1817. doi: 10.1016/j.jpedsurg.2016.07.015. Epub 2016 Aug 5.

Abstract

Introduction: Colorectal cancer (CRC) diagnosed before age 30 years is a fatal disease whose biology remains poorly understood. To understand its pathogenesis, we compared molecular and clinical data in surgically treated early-age onset and adult onset patients.

Materials and methods: Clinical data and tumor tissue were collected retrospectively for 94 patients with early-age onset CRC (age ≤30 years) and compared to 275 adult CRC patients (age ≥50 years). Tumor morphology, microsatellite instability (MSI) and stability (MSS), KRAS and BRAF mutations, and mismatch repair (MMR) expression (MSH2, MLH1, MSH6, PMS2) were assessed.

Results: Early-age CRC was distinguished from adult CRC by advanced stage presentation (P<0.001), frequent high grade cancers (P<0.001), and poor prognosis (P<0.001). MSI was associated with favorable survival and MMR loss in both groups. Compared to adults, MSI in early-onset CRC was more prevalent (P<0.01), not tightly linked to MLH1/PMS2 loss, and never associated with BRAFV600E mutations (P<0.01). MSS/BRAFV600E genotype had poor prognosis and was more prevalent in early-age CRC (9% vs. 3%).

Discussion: Specific genetic subtypes are found at different frequencies in early-age onset and adult onset CRC. Complete absence of the indolent MSI/BRAFV600E genotype and enrichment in the unfavorable MSS/BRAFV600E genotype help explain the poor prognosis of early onset CRC.

Keywords: BRAF; Early onset colorectal cancer; MSI.

MeSH terms

  • Adaptor Proteins, Signal Transducing / genetics*
  • Adaptor Proteins, Signal Transducing / metabolism
  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / metabolism
  • Child
  • Colorectal Neoplasms / diagnosis*
  • Colorectal Neoplasms / genetics
  • Colorectal Neoplasms / mortality
  • DNA Mismatch Repair
  • DNA Mutational Analysis
  • DNA, Neoplasm / genetics*
  • Female
  • Humans
  • Male
  • Microsatellite Instability
  • Middle Aged
  • Mutation*
  • Neoplasm Staging*
  • Retrospective Studies
  • Survival Rate / trends
  • United States / epidemiology
  • Young Adult

Substances

  • Adaptor Proteins, Signal Transducing
  • Biomarkers, Tumor
  • DNA, Neoplasm