Journal of Pediatric Surgery Open (Apr 2023)
Pediatric rectal cancer: Diagnosis and management of a rare problem
Abstract
Introduction: Colorectal carcinoma represents only 1% of all pediatric malignancies, thus, little is known about its pathogenesis. Among these small number of cases, rectal carcinoma singularly is even more rare, and often studied in conjunction with colon carcinoma. We compared demographic and therapeutic data of pediatric rectal carcinoma cases to better characterize the malignancy in children. Methods: A retrospective cohort of 8,721,474 tumors from the Surveillance, Epidemiology, and End Results (SEER) database consisting of years 2000-2019 was divided by gender, race, site of presentation, and five pediatric age groups: infant (>1 years), toddler (1-4 years), child (5-9 years), adolescent (10-14 years), and teenager (15-19 years). We further explored the efficacy of therapeutic approaches by determining the mortality in patients who underwent surgical resection. Results: From 2000 to 2019, there were 1020 cases of children 0-19 with cancer at colorectal sites, of which, 8.6% (n= 89) of cases were at the rectum and rectosigmoid junction only. All cases were found in children above 10 years of age, with the earliest cases presenting in the 10–14-year age group. 58% (n= 52) and 42% (n= 37) of cases were in males and females respectively. 73% (n= 65) of cases were identified in Caucasian patients followed by 12% (n= 11) in Black patients. 60.7% (n= 54) of malignant rectal cancer cases were adenocarcinomas. The overall mortality rate of pediatric rectal carcinoma was 37.1% (n= 33). Regarding therapy, 68 patients (76%) received resectional surgery alone. Mortality in patients who received neo/aduvant radiation therapy with surgery was 56% (n= 14) and 57.6% (n= 19) in those who received chemotherapy alone. Among patients who received beam radiation and surgery, 76%% (n= 19) received therapy prior to surgery, 16% (n= 4) received therapy after surgery and 8% (n= 2) received radiation therapy before and after surgery. Conclusions: We found that pediatric rectal carcinoma was more common with increasing age and in Caucasian children. Most cases were malignant adenocarcinomas, with treatment options yielding poor outcomes. Advanced presentations not amenable to surgical treatment or needing preoperative radiation are seen in nearly a third of pediatric rectal cancers. The rarity of this malignancy along with the fact that clinical symptoms (such as constipation) are frequent in pediatric patients, delays the time between symptom onset and diagnosis. Increasing our knowledge on the presentation of pediatric rectal carcinoma and how we can optimize our therapeutic approach can improve screening protocols and outcomes.
