BACKGROUND: Since the first description of a pancreatoblastoma as a malignant pancreatic tumour of childhood in 1957, approximately 60 cases have been reported. We present the symptoms, pathology, and therapy of this rare tumour in a 17-year-old girl. CASE REPORT: The patient initially presented with upper gastro-intestinal bleeding. During laparotomy a 16-cm (diameter) tumour was recognized in the pancreatic tail. Open biopsy and radical resection, followed by histological and immunohistochemical examinations, confirmed the diagnosis of a pancreatoblastoma. The results of single-cell DNA cytometry underlined the low DNA grade of malignancy of the primary tumour. In spite of adjuvant chemotherapy with cisplatin and adriamycin, the patient returned 22 months later with many hepatic and peritoneal metastases measuring up to 5 cm in diameter. Peritoneal tumour debulking and an extended hemihepatectomy were performed as a palliative treatment. Unfortunately, the patient died 18 months later from further tumour progression. CONCLUSIONS: A review of the literature reveals that a pancreatoblastoma in childhood has to be considered malignant, but usually shows a favourable prognosis in contrast to pancreatic neoplasms in adult patients. The treatment of choice is radical resection. Adjuvant chemotherapy or radiotherapy should be considered because of the metastatic potential of the tumour.
BACKGROUND: Since the first description of a pancreatoblastoma as a malignant pancreatic tumour of childhood in 1957, approximately 60 cases have been reported. We present the symptoms, pathology, and therapy of this rare tumour in a 17-year-old girl. CASE REPORT: The patient initially presented with upper gastro-intestinal bleeding. During laparotomy a 16-cm (diameter) tumour was recognized in the pancreatic tail. Open biopsy and radical resection, followed by histological and immunohistochemical examinations, confirmed the diagnosis of a pancreatoblastoma. The results of single-cell DNA cytometry underlined the low DNA grade of malignancy of the primary tumour. In spite of adjuvant chemotherapy with cisplatin and adriamycin, the patient returned 22 months later with many hepatic and peritoneal metastases measuring up to 5 cm in diameter. Peritoneal tumour debulking and an extended hemihepatectomy were performed as a palliative treatment. Unfortunately, the patient died 18 months later from further tumour progression. CONCLUSIONS: A review of the literature reveals that a pancreatoblastoma in childhood has to be considered malignant, but usually shows a favourable prognosis in contrast to pancreatic neoplasms in adult patients. The treatment of choice is radical resection. Adjuvant chemotherapy or radiotherapy should be considered because of the metastatic potential of the tumour.