Primary Pediatric Gastrointestinal Tract Lymphoma: A Review of 265 Patients

Objective: To determine incidence, outcomes, and predictors of survival of primary gastrointestinal tract lymphoma (PGTL) in pediatric patients. Methods: The SEER database was queried from 1973-2005 for all patients under 20 years of age. Results: Overall, 265 patients were identified with PGTL for an annual age adjusted to year 2000 incidence of 0.199 cases per 100,000. Most patients were white (89%), non-Hispanic (82%), and male (77%). Tumors were most commonly identified in the small bowel (55%), colon (33%), and stomach (9%), but rarely in the esophagus (0.4%) or rectum/anus (1.5%). Burkitt lymphoma was the most common histology type (53%). Disease-specific 5 and 10-year survival for the entire cohort was 84% and 82% respectively. By univariate analysis, tumors located in the stomach had the worst 10-year survival while tumors located in the rectum or anus had the best survival (59% vs. 100%, p=0.03). No difference in 10-year survival was observed in patients who had surgical resection (81% vs. 94%, p=0.076). Patients who received radiation therapy had worse 10-year survival (65% vs. 84% for no radiation, p=0.004). By multivariate analysis, tumor location was an independent predictor of mortality [small bowel (HR 0.197, p=0.002) and large bowel (HR 0.222, p=0.006)]. Poorly differentiated lymphoma carried a poor prognosis (HR 3.867, p=0.036). No difference in survival was observed for those who had surgery for treatment (p=0.099) or received radiation (p=0.713). Conclusion: PGTL most commonly involves the small bowel and colon, which are favorable survival tumor locations. Surgery for treatment does not increase survival in children with PGTL.