Original Article
Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database

https://doi.org/10.1016/j.jpedsurg.2015.11.005Get rights and content

Abstract

Background

Pediatric colorectal cancer (CRC) is rare. Comparison with adult CRC tumors, management, and outcomes may identify opportunities for improvement in pediatric CRC care.

Study Design

CRC patients in the National Cancer Data Base from 1998 to 2011, were grouped into Pediatric (≤ 21 years), early onset adult (22–50) and older adult (> 50) patients. Groups were compared with χ2 and survival analysis.

Results

A total of 918 pediatric (Ped), 157,779 early onset adult (EA), and 1,304,085 older adults (OA) were identified (p < 0.01 for all comparisons). Patients ≤ 50 presented more frequently with stage 3 and 4 disease (Ped: 62.0%, EA: 49.7%, OA: 37.3%) and rectal cancer (Ped: 23.6%, EA: 27.5%, OA: 19.2%). Pediatric histology was more likely signet ring, mucinous, and poorly differentiated. Initial treatment was usually surgery, but patients ≤ 50 were more likely to have radiation (Ped: 15.1%, EA: 18.6%, and OA: 9.2%) and chemotherapy (Ped: 42.0%, EA: 38.2%, and OA: 22.7%). Children and older adults showed poorer overall survival at 5 years when compared to early onset adults. Adjusting for covariates, age ≤ 21 was a significant predictor of mortality for colon and rectal cancers (colon HR: 1.22, rectal HR: 1.69).

Conclusions

This is the largest cohort of pediatric CRC patients, revealing more aggressive tumor histology and behavior in children, particularly in rectal cancer. Despite standard oncologic treatment, age ≤ 21 was a significant predictor of mortality. This is likely owing to worse tumor biology rather than treatment disparities and may signal the need for different therapeutic strategies.

Section snippets

Methods

Ethical approval for this study was granted by the Maine Medical Center Institutional Review Board. All patients with a histologic diagnosis of colorectal adenocarcinoma, using ICD-O-3 classification, diagnosed between years 1998 and 2011 were identified from the NCDB. The NCDB, a joint project of the American Cancer Society and the Commission on Cancer (CoC) of the American College of Surgeons, is a nationwide, facility-based, comprehensive clinical surveillance resource oncology data set that

Demographics (Table 1)

A total of 918 pediatric (Ped), 157, 577 early onset adult (EA), and 1,303,655 older adult (OA) patients were identified (Fig. 1). There were no significant differences in gender or race among the 3 age groups. Comorbidities, as measured by the Charlson Deyo score, increased with increasing age. Significant differences exist in presenting location of tumor, with older adults presenting with a higher proportion of right colon cancer (Ped: 23.7%, EA: 22.9%, OA: 35.8%, p < 0.001) whereas the early

Discussion

To our knowledge, the current study represents the largest cohort (N = 918) of pediatric colorectal cancer patients studied. These results reveal poorer survival in children, even when adjusted for known prognostic factors such as stage, pathology, and behavior. Notably, the mortality risk associated with the pediatric age group was affected by the tumor location, with a greater effect on mortality for rectal tumors. This study also confirms findings from prior studies demonstrating more

Conclusions

To our knowledge, this study represents the largest cohort of pediatric CRC patients and confirms more aggressive tumor histology and behavior in children, even with standard oncologic treatment in younger patients. This presentation is associated with a higher 5 year mortality in pediatric patients when compared to the adult cohorts. Poorer outcomes in pediatric CRC patients appear to be owing to worse tumor biology and stage rather than treatment disparities. Furthermore colon and rectal

Acknowledgment

Jeremy Kane, PhD for help with statistical analysis.

References (36)

  • D.P. Winchester et al.

    The national cancer data base: past, present, and future

    Ann Surg Oncol

    (2010)
  • M.T. Sharabiani et al.

    Systematic review of comorbidity indices for administrative data

    Med Care

    (2012)
  • M. Gönen et al.

    Concordance probability and discriminatory power in proportional hazards regression

    Biometrika

    (2005)
  • D. Kravarusic et al.

    Colorectal carcinoma in childhood: a retrospective multicenter study

    J Pediatr Gastroenterol Nutr

    (2007)
  • L. Losi et al.

    Molecular genetic alterations and clinical features in early-onset colorectal carcinomas and their role for the recognition of hereditary cancer syndromes

    Am J Gastroenterol

    (2005)
  • E.R. Fearon

    Human cancer syndromes: clues to the origin and nature of cancer

    Science

    (1997)
  • H.C. Kim et al.

    Methylation of the hMLH1 and hMSH2 promoter in early-onset sporadic colorectal carcinomas with microsatellite instability

    Int J Colorectal Dis

    (2003)
  • C. Durno et al.

    Family history and molecular features of children, adolescents, and young adults with colorectal carcinoma

    Gut

    (2005)
  • Cited by (45)

    • CDH1 gene mutation in early-onset, colorectal signet-ring cell carcinoma

      2020, Pathology Research and Practice
      Citation Excerpt :

      Collectively, these studies support anecdotal evidence of high rates of colorectal SRCC in young Pakistani people. Studies suggest the younger the age of the patient, the poorer the outcome [19,43]. The youngest patient in the cohort was only eight years old and 35 % (7 of 20) of the patients diagnosed with CRC with signet ring cell component (irrespective of whether signet ring cells were major (>50 %) or minor (<50 %) component of the tumour) were younger than 22 years of age, a cut-off which has been used to define CRC patient populations as paediatric (≤21 years) and early-onset adult (22–50 years), respectively [43].

    • Colon Cancer in Patients Under 25 Years Old: A Different Disease?

      2020, Journal of the American College of Surgeons
      Citation Excerpt :

      Because we found no difference in adjuvant chemotherapy or radiation therapy, this NCDB study found that although initial treatment was usually surgery, patients aged 50 years or younger were more likely to have radiation (Ped: 15.1%, EA: 18.6%, and OA: 9.2%) and chemotherapy (Ped: 42.0%, EA: 38.2%, and OA: 22.7%) (colon HR 1.22, rectal HR 1.69). On multivariate analysis, age less than or equal to 21 years was a significant predictor of mortality for colon and rectal cancer.14 This is the first time peritoneal metastasis has been shown to be statistically significantly more prevalent in pediatric patients with colon cancer.

    View all citing articles on Scopus
    View full text