Pediatric Colorectal Cancer Epidemiology and Treatment
Summary
Pediatric colorectal carcinoma is rare, accounting for less than 1% of new childhood malignancies globally. Incidence has shown a small but steady rise among adolescents, with a peak in mid‐teens. A greater proportion of tumours in this population present at an advanced stage, often with distant metastases at diagnosis, reflecting both aggressive biology and diagnostic delay due to nonspecific symptoms. Histologically, mucinous adenocarcinoma and signet ring cell carcinoma predominate, contrasting with the adenomatous adenocarcinomas more common in adults. A substantial subset harbour germline or sporadic defects in DNA mismatch repair machinery, resulting in microsatellite instability and a hypermutated genotype. Management principles derive from adult CRC protocols with adaptations: complete surgical excision remains the cornerstone of curative intent, often supplemented by adjuvant chemotherapy and, in rectal primaries, radiotherapy. Emerging evidence supports the integration of neoadjuvant immunotherapy for mismatch‐repair‐deficient tumours, achieving high rates of complete pathological response in selected cases. Multidisciplinary care, genetic counselling and long‐term surveillance are essential to address inherited predispositions and optimise outcomes.
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Pediatric Colorectal Cancer Epidemiology and Treatment publication trend
The graph below shows the total number of articles in pediatric colorectal cancer epidemiology and treatment across all publications each year (not limited to Nature Index journals).
Technical terms
Microsatellite instability-high (MSI-H): A genomic phenotype characterised by frequent insertion–deletion mutations in short tandem DNA repeats due to defective mismatch repair.
Deficient mismatch repair (dMMR): Loss of function in DNA repair proteins, leading to accumulation of replication errors and hypermutability.
Mucinous adenocarcinoma: A subtype of colorectal cancer defined by abundant extracellular mucin comprising more than 50% of the tumour volume.
Signet ring cell carcinoma: A form of carcinoma in which malignant cells contain intracytoplasmic mucin that displaces the nucleus to the cell periphery.
Neoadjuvant immunotherapy: Administration of immune-modulating agents prior to surgical resection to enhance antitumour response.
R0 resection: Surgical removal of a tumour with histologically negative margins, indicating no residual microscopic disease.
References
- Clinical, Pathology, Genetic, and Molecular Features of Colorectal Tumors in Adolescents and Adults 25 Years or Younger. Clinical Gastroenterology and Hepatology (2020).
- Pathological complete response after neoadjuvant immunotherapy combined with chemotherapy in pediatric rectal carcinoma: A case report. Frontiers in Immunology (2022).
- Colorectal cancer under 20 years old: a retrospective analysis from three tertiary hospitals. Journal of Cancer Research and Clinical Oncology (2020).
- Insights from a retrospective study: an understanding of pediatric colorectal carcinoma. Egyptian Pediatric Association Gazette (2024).
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