Objective: To analyze clinical features, outcomes and correlative prognostic factors of the disease.
Method: Totally 14 newly diagnosed children with pancreatoblastoma at Shanghai Children's Medical Center, Children's Hospital of Chongqing Medical University, Sun Yat-sen University Cancer Center and Beijing Children's Hospital were enrolled into this study between April 2003 and July 2013. The clinical features, surgery, chemotherapy protocol and prognostic factors of patients were retrospectively analyzed.
Result: The median age at diagnosis was 6.5 years (0.6 to 11 years). Four cases had local tumor, 9 cases had local invasion, and in 1 case lung and liver metastasis was found at diagnosis. Increased serum α-fetoprotein were found in 8 cases (66-54,000 μg/L). In 8 patients the tumor was resected at first. The remaining 6 patients had biopsy and in four of them the tumor was completely resected after 4-6 cycles of chemotherapy. Except for 2 patients who gave up treatment, all the other patients fulfilled the comprehensive treatment including surgery and chemotherapy. One patient received radiofrequency ablation. The median follow-up period was 26 months(16-140 months). The estimated 2 year event free survival was (66.7 ± 13.6)%.On univariate analysis, non-resectable tumor(χ(2) = 6.663, P = 0.010), progression and metastases during treatment(χ(2) = 14.927, P = 0.000), poor response to treatment(χ(2) = 12.293, P = 0.000)were independent predictors for poor prognosis.
Conclusion: Complete resection and complete remission after treatment were excellent prognostic factors. Some initially unresectable disease seems to be resectable after adjuvant chemotherapy, which may be a key point of treatment strategy.