Epidemiology and management options for colorectal cancer in children.

Colorectal carcinoma (CRC), although primarily a disease of adulthood, accounts for 2% of malignancies in adolescents and has been reported in children as young as 9 months of age. Our knowledge of CRC in pediatrics is based on a handful of case series and case reports. Apart from one small clinical trial, there has been a lack of prospective clinical studies in this age group. Based on these published reports, most CRC in children is sporadic, but it can also arise in the setting of predisposing conditions, such as gastrointestinal polyposis syndromes, nonpolyposis familial cancer syndromes, and inflammatory bowel disease. Despite some similarities to adult disease, CRC in childhood may be intrinsically different biologically, because it differs from adult-onset CRC in several respects. Childhood CRC tends to be diagnosed at an advanced stage, is largely of mucinous histology, and (probably because of these features) tends to have a poorer outcome. As a result of its rarity in children and the lack of prospective pediatric studies, recommendations for therapy are primarily extrapolated from adult clinical trials. A review of pediatric case series in the English literature emphasizes the prognostic significance of stage of disease, as well as extent of surgical resection. As in adults, early detection is critical in an effort to capture the disease at less advanced stages. Complete surgical resection with aggressive lymph node dissection is essential for cure, and neoadjuvant chemotherapy may be used in an effort to render unresectable lesions resectable. Active agents in adults with CRC include fluorouracil, folinic acid (leucovorin), oxaliplatin, and irinotecan. Furthermore, newer targeted therapeutic agents, such as bevacizumab and cetuximab, have added additional efficacy to the standard chemotherapy backbone. Collaborative multi-institutional pediatric clinical trials are needed to evaluate the prognosis, optimal treatment response, and the basic biology of childhood onset CRC.