Local Control Efficacy of Radiotherapy and Prognostic Factors in Pancreatoblastoma: A Single-Center Experience With a Rare Pediatric Tumor.
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PURPOSE: To investigate the patterns of recurrence/metastasis and the clinical value of radiotherapy in local control for pediatric pancreatoblastoma. MATERIALS AND METHODS: A retrospective analysis was conducted on 14 pediatric patients with pathologically confirmed pancreatoblastoma treated at our institution from June 2017 to June 2024. Clinical data, including baseline characteristics, surgical approaches, pathological staging, adjuvant therapies (chemotherapy/radiotherapy), recurrence/metastasis patterns, and subsequent interventions, were systematically collected. The impact of radiotherapy on local control was evaluated, with survival analysis performed using Kaplan-Meier methods, and prognostic factors analyzed via log-rank tests and Cox regression models. RESULTS: The median age of the entire cohort was 7 years (range, 3-13 years), with 4 cases of pancreatic head tumors and 10 cases of pancreatic body/tail tumors. At initial diagnosis, 57.1% (8/14) presented with regional lymph node metastasis, and 57.1% (8/14) had distant metastasis. The R0 resection rate during the first surgery was 57.1% (8/14), while R1/R2 resections accounted for 28.6% (4/14); 2 did not undergo surgery. With a median follow-up of 31 months, the overall survival rate was 78.6% (11/14). The recurrence/metastasis rate was 64.2% (9/14), with predominant patterns including tumor bed recurrence (3/9, 33.3%), regional lymph node metastasis (3/9, 66.7%), and liver metastasis (5/9, 55.6%). Multimodal therapies encompassed chemotherapy, secondary surgery, liver transplantation, and radiotherapy for metastatic lesions. In the radiotherapy group, the 1-year and 2-year local control rates were 100% and 88%, respectively. Log-rank test and Cox analysis identified failure to achieve R0 resection and regional lymph node metastasis as independent prognostic factors for inferior overall survival (P < 0.05). Other factors-including age, gender, presence of initial metastasis, initial liver/lung metastasis, number of recurrence/metastasis events, and radiotherapy-showed no significant correlation with overall survival. CONCLUSION: Regional lymph node metastasis and failure to achieve R0 resection are critical prognostic factors affecting long-term survival in pancreatoblastoma patients. Adjuvant radiotherapy significantly improves local control rates and may enhance survival outcomes in patients with positive margins or lymph node metastasis by strengthening local disease control, warranting further validation in prospective studies.