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

Abstract

Introduction: 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.

Patients and methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Adolescents; Children; Colorectal adenocarcinoma; Surgery.

Fig. 1

Abdominal CT-scan image demonstrating neoplastic mass in the descending colon which measures 5 × 3 cm

Fig. 2

Peritoneal carcinosis observed at laparoscopy

Fig. 3

Signet-ring cell carcinoma effacing the colonic wall structure (hematoxylin and eosin, original magnification 40×) with high magnification insert showing signet-ring cells originating from the mucosa and infiltrating the underlying stroma (hematoxylin and eosin, original magnification 300×)