Colorectal adenocarcinoma in children and adolescents: the management of advanced disease

TL;DR

Colorectal adenocarcinoma in children is rare and aggressive, with poor outcomes due to delayed diagnosis and limited treatment effectiveness.

Contribution

The paper presents a case series of three pediatric patients with advanced colorectal adenocarcinoma and highlights management challenges.

Findings

Pediatric colorectal adenocarcinoma is rare and often diagnosed at advanced stages.

Two out of three patients in the case series died within a year after surgery.

Laparoscopic resection was used to manage intestinal obstruction without creating stomas.

Abstract

Colorectal carcinoma (CC) is a rare disease in the pediatric population, with an annual incidence of 1 in 10 million adolescents, and it accounts for approximately 1% of pediatric solid neoplasms. It is the most common primary gastrointestinal malignancy in children with the vast majority of CCs being adenocarcinoma (CA). Unfortunately, the proportion of poorly differentiated, mucinous type, signet-ring cell containing carcinomas is higher in younger patients than in adults. Moreover, due to the low awareness of the disease, diagnosis is usually delayed until advanced stages, resulting in an extremely poor prognosis. Surgery is the only curative modality for localized CAs, whereas adjuvant chemotherapy is the standard of care for patients with stage III cancer to eradicate micro-metastases.

In the last 10 years, we treated 3 patients diagnosed with CA: a 14-year-old female, a 15-year-old male, and a 15-year-old female. All patients presented to our Emergency Department with nonspecific symptoms of abdominal pain and vomiting.

All patients were subjected to laparoscopic tumor resection to relief intestinal obstruction. In the male patient, laparoscopy was converted to laparotomy to safely assess the anatomy because of strong peritoneal adhesions. No stomas were created, in order to improve quality of life. Oxaliplatin and 5-fluorouracil-based regimens were among the most commonly used chemotherapy combinations. The 15-year-old female and the 15-year-old male died 1 year after the surgical resection, while the 14-year-old female is still on follow-up.

CAs behave aggressively in children; they not only show a poorer response to chemotherapy, but are also associated with extensive intramural spread and peritoneal carcinomatosis. Lack of awareness and timely intervention remain the main challenges for early diagnosis and improved prognosis of CA.

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