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Background  Whether surgery can improve the prognosis of patients with primary pediatric gastrointestinal lymphoma (PPGL) who experienced bowel perforation remains controversial. This study aimed to evaluate the prognosis of such patients.    Methods  Nine patients pathologically diagnosed with PPGL who experienced perforation at our center between January 2010 and December 2020 were enrolled and divided into two groups: those with perforation during ( n  = 4) and before ( n  = 5) chemotherapy. Their medical records were reviewed, and long-term follow-up was conducted by telephone in February 2021.    Results  All patients with perforation during chemotherapy were diagnosed with PPGL in the outpatient department. The mean time from outpatient visit to chemotherapy was 17.3 ± 6.1 days. Two patients experienced perforation during the first chemotherapy regimen and received conservative treatment, while the others developed perforation after multiple chemotherapy regimens and underwent surgery. All of the patients received regular chemotherapy and survived for a mean follow-up time of 3.8 ± 1.9 years. No patient with perforation before chemotherapy had a definite diagnosis in the outpatient department. Among these patients, 4 experienced perforation and underwent surgery, of whom 3 developed perforation-related complications and died; the other recurred after chemotherapy. Only the patient who received conservative treatment was diagnosed with PPGL before chemotherapy, received regular chemotherapy, and survived without a recurrence for 1.0 year.    Conclusion  Prompt diagnosis and chemotherapy improve the prognosis of PPGL. Surgery does not affect the prognosis of patients with perforation during chemotherapy but may accelerate disease progression in patients with perforation before chemotherapy.

Clinical features and outcomes of bowel perforation in primary pediatric gastrointestinal lymphoma