Solid-pseudopapillary tumor of the pancreas and pancreatoblastoma in children: Experience over 18 years at a Pediatric Oncology Center

Abstract

9062 Background: Malignant pancreatic tumor is a rare disease in the pediatric population. Guidelines for treatment, including management in advanced disease are controversial until this moment (REBHANDL, 2001). Regarding to the pathology findings, pancreatoblastoma and solid-pseudopapillary tumor of the pancreas (Frantz’s tumor) are the more frequent pancreatic tumors in childhood. The aim of this study was a retrospective analysis of all malignant pancreatic tumors diagnosed at Boldrini’s Children Cancer Center during a period of 18 years. Methods: A retrospective analysis of eight cases of malignant pancreatic tumors, admitted at the Boldrini’s Children Cancer Center, from January 1987 to April 2005. All the data concerning age, sex, clinical presentation, stage, treatment and follow-up were collected. Results: The patient’s population refers to 8 children. There were 4 boys and 4 girls. Age ranged from 1 month to 15 years old (median age of 14 years). Main symptom was the increase of abdominal volume. None of them had metastatic disease at diagnosis. Regarding to pathology, 4 patients had pancreatoblastoma and 4 Frantz’s tumor. One patient (who had pancreatoblastoma) received chemotherapy before surgery, with a partial response, and then had a complete tumor ressection. All others (7 patients) had complete surgery as first treatment. Only one patient with Frantz’s tumor had pancreatic injury during tumor ressection and is now receiving enzimatic reposition. All the patients are alive, free of disease, from 6 months to 11 years (median follow-up of 2 years). Table I . Conclusions: Total pancreatectomy is the most important treatment in pancreatoblastoma and Frantz’s tumor. Neoadjuvant chemotherapy may be an important tool in unresectable tumors, increasing the disease-free survival. [Table: see text] No significant financial relationships to disclose.