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Clinical implications and surgical management of intussusception in pediatric patients with Burkitt lymphoma
Himesh Gupta1, Andrew M Davidoff, Ching-Hon Pui
1Department of Surgery, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
Insights
Pediatric Burkitt lymphoma presenting with intussusception often indicates early-stage disease, with most patients achieving complete resection and long-term remission. This presentation is linked to curable, less intensive treatment outcomes in children.
Area of Science:
- Pediatric oncology
- Surgical oncology
- Gastroenterology
Background:
- Intussusception is a rare presenting symptom of Burkitt lymphoma.
- Early-stage Burkitt lymphoma is curable with less intensive therapy.
Purpose of the Study:
- To determine the incidence, presentation, stage, and outcomes of pediatric Burkitt lymphoma cases presenting with intussusception.
Main Methods:
- Retrospective review of medical records from 1962 to 2005.
- Analysis of patients with Burkitt lymphoma who presented with intussusception.
Main Results:
- 17.5% (33/189) of pediatric abdominal Burkitt lymphoma cases presented with intussusception.
- Patients with intussusception had higher rates of complete tumor resection (70%) and lower stage disease (Stage II).
- 25 patients achieved long-term remission (median 14 years).
Conclusions:
- Pediatric Burkitt lymphoma with intussusception is often resectable and associated with favorable outcomes.
- Patients presenting with intussusception tend to be older than the general pediatric population with intussusception.
Background/Purpose:
Intussusception as a presenting feature of Burkitt lymphoma may be associated with early stage disease, which is curable with less intensive therapy. We determined the incidence, presentation, stage, and outcome of children with Burkitt lymphoma presenting with intussusception.
Methods:
The medical records of patients with Burkitt lymphoma treated at our hospital from 1962 to 2005 were reviewed, and the patients presenting with intussusception were then further analyzed.
Results:
Of 189 patients with primary abdominal Burkitt lymphoma, 33 (17.5%) presented with intussusception. Their median age at diagnosis was 10 years (range, 3-19 years). Most patients presented with abdominal pain (88%) and/or nausea/vomiting (42%). Twenty-three (70%) of these 33 patients as compared with 10 of the other 156 patients with abdominal lymphoma could have complete resection of their tumor (P < .0001) and hence had low stage disease (stage II). Only 10 patients with intussusception had stage III (n = 7) or stage IV (n = 3) unresectable disease. Twenty-five of the patients remained alive in continuous complete remission for 3 months to 31 years (median, 14 years).
Conclusion:
Pediatric patients with Burkitt lymphoma presenting with intussusception often have completely resectable disease and are older than general pediatric patients with intussusception.
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