Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center.

Background: Pancreatoblastoma is a very rare malignant pancreatic tumor in children. Pancreatoblastoma is the most common pancreatic tumor in children less than 10 years of age, accounting for 25% of the pancreatic neoplasm. There were only a few published literatures about the standardized diagnostic and management protocol for PB in the last decade.

Objective: To summarize our experience in the management of pancreatoblastoma in children and adolescents with emphasis on the presentation, diagnosis, treatment, and outcomes. A management strategy will also be discussed.

Methods: This was a retrospective case-series study of all pancreatoblastoma in patients < 18 years of age who were treated at Beijing children's hospital (BCH) from January 2002-January 2015. The diagnoses of PB were confirmed by histopathology analysis of the resected specimen. The variables being analyzed included patient demographics, age at diagnosis, clinical presentation, tumor size, metastasis if present, tumor markers (AFP), type of surgery, length of follow-up, and outcome. The assessment of the tumor location, size, extent of the tumor, and distant metastasis was made by ultrasound (US), computed tomography (CT), and/or magnetic resonance imaging (MRI).

Result: 21 patients with pancreatoblastoma were diagnosed at a median age of 4 years, 7 girls, and 14 boys. The diagnosis of pancreatoblastoma was identified by the histology examination. The most common syndrome was abdominal mass (n = 11), followed by abdominal pain (N = 10), elevated serum AFP levels were noted in almost all cases (17/18), 17 patients with disease initially unresectable on diagnosis accepted neo-adjuvant chemotherapy consisting of CDV, OPEC, PLADO, IEV, and AVCP. All patients underwent surgery, including pancreaticoduodenectomy (Whipple's procedure), the Pylorus-preserving pancreaticoduodenectomy (traverse-Longmire procedure), Spleen-preserving distal pancreatectomy, and distal pancreatectomy with en bloc splenectomy, Roux-en-Y end-to-end pancreatojejunostomy. In all, 13 children were disease free with a median follow-up of 53 months (range 11-156 months).

Conclusions: The pancreatoblastoma in children and adolescents is a malignant tumor. Complete resection combined with chemotherapy is associated with long-term survival. For the unresectable tumor at diagnosis, preoperative chemotherapy was recommended to reduce tumor volume. AFP is critical for diagnosis and monitoring the disease as a tumors marker.