Abstract

Introduction:Pancreatoblastoma (PB) is a rare pancreatic neoplasm that occurs most in pediatric patients. Here, we report a rare case of adult PB with liver metastasis and review the literature in order to assist clinicians in the management of the disease.Case Presentation:A 27-year-old female patient suffered from postprandial fullness, anorexia, and weight loss in the past 3 months. An abdominal ultrasound and contrast-enhanced computed tomography scans confirmed right abdominal mass with compression of major liver vessels, as well as the P-duct and biliary ducts and causing mild dilatation. Pancreatoduodenectomy was performed and pathologic findings showed typical squamoid corpuscles, which confirmed the diagnosis of PB. The patient was alive and disease-free for 1 year and 10 months until a new metastatic lesion was found. Radiofrequency ablation was arranged as a curative treatment, and no viable tumor or sign of recurrence was found until this paper was submitted. Based on a review of previous case reports, we found adult PB patients with only liver metastasis presented with a smaller tumor size (P = 0.031), more frequent pancreatic head origin (P = 0.043), and decreased 1-year mortality (P = 0.009) compared with patients with other distal metastases. Therefore, we assumed that PB with liver metastasis might present favorable outcome by complete surgical resection or other curative treatment.Conclusion:PB patients with liver metastasis are more likely to show a pancreatic head origin, smaller tumor size, and more favorable outcomes compared with other sites of metastasis. PB should be treated aggressively with surgical resection or other curative treatment as opposed to chemotherapy alone.

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