Poor outcome of gastrointestinal perforations associated with childhood abdominal non-Hodgkin's lymphoma

Background/Purpose: With modern chemotherapeutic protocols and advances in medical care, the outcome of intraabdominal non-Hodgkin's lymphoma (NHL) in children can be excellent for limited disease. Advanced disease, however, is associated with increased tumor aggression and requires more rigorous adjuvant therapy. Hence, complications early in the course of the disease process or its management often lead to a poor outcome. Perforation of the gastrointestinal tract, either iatrogenic, tumor related or chemotherapeutically induced is one such complication and may result significant morbidity and mortality. Methods: The authors reviewed their experience with this disease, and present two cases of children with abdominal NHL, which poignantly demonstrate these points. Results and a review of the literature are then discussed. Results: Fifteen cases of abdominal NHL were examined with an overall mortality rate of 40%. This increased to 100% in the presence of perforation. In two cases, inadvertent entry into the bowel occurred at the time of laparotomy for tumor biopsy. In the first case, intestinal wall was included in the biopsy specimen; in the second, laparotomy unmasked an already sealed-off perforation secondary to tumor invasion. Sepsis ensued in both cases. In the first, this resulted in repeated delays in chemotherapy, and the child succumbed to the disease. In the second, chemotherapy was continued, and although the small bowel leak was controlled, the initial insult hampered marrow recovery and host defenses, resulting in fatal sepsis. Conclusions: These data and other reported cases in the literature indicate that intestinal perforation associated with abdominal lymphomas in children portends an extremely poor prognosis. All attempts to avoid this complication should be made, including avoiding direct tumor biopsy whenever possible.