ABSTRACT
Introduction
Colorectal carcinoma (CRC) is one of the most common tumors in adult, but is extremely rare in children. In childhood, CRC often presents unfavorable aggressive histotypes, advanced clinical stage at onset and a worse prognosis. Pediatric CRC series are limited and include few patients, therefore information about treatment strategy and pharmacotherapy is scarce. For this reason, management of these patients represents a real challenge for pediatric oncologists.
Areas covered
The authors provide an overview of the general features and management strategies of pediatric CRC with specific attention to systemic treatment. Literature data regarding pharmacotherapy in published pediatric series are summarized and analyzed in detail, according to adult treatment standards.
Expert opinion
In the absence of specific recommendations for pediatric CRC, the general therapeutic strategy should follow the same principles as for adults and should be the result of a multidisciplinary discussion. Patient access to optimal treatment is difficult due to the lack of new drugs approved for the pediatric age group and non-availability of clinical trials. Collaboration between pediatric and adult oncologists is considered crucial in order to overcome these issues and find solutions to increase knowledge and improve the outcome of such a rare disease in children.
Article highlights
Colorectal carcinoma (CRC) is a very rare tumor in childhood with peculiar features. It is characterized by a higher incidence of aggressive histotypes and advanced clinical stages at onset than in adults and the prognosis is poor.
Multiple series on CRC in young age have been published through the years however the number of children and adolescent patients included is limited. Only few of these reported detailed data about systemic treatment.
The lack of experience and literature data did not allow to develop specific recommendations for pediatric CRC, so the therapeutic strategy should follow the same principles used for adults and should be defined through multidisciplinary discussion. Surgery only for localized disease, neoadjuvant/adjvuvant chemotherapy for locally advanced disease, and systemic drugs combinations for metastatic disease are the mainstay of the treatment also in childhood.
The almost absence of dedicated clinical trials for pediatric CRC and difficulties in the access to new agents make the management of this disease really challenging. The activity of International cooperative working groups and the collaboration with adult oncologists are fundamental to offer to these patients the best treatment.
Further research with new retrospective analyses, prospective data, and molecular studies should be promoted to increase the knowledge on pediatric CRC and optimize the treatments to improve outcome.
Declaration of interest
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties
Reviewer disclosures
Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.