[Pancreatoblastoma--a rare pancreatic malignancy in childhood]

Between 1885 and 1991 only 71 cases of malignant pancreatic tumours in childhood and adolescence have been reported in literature; the majority of these were pancreatoblastomas. The symptoms, pathology and therapy of this rare tumour are demonstrated in the case of a 17-year-old girl. The tumour is believed to develop at an early stage of pancreatic cell differentiation. Usually it is composed of both exocrine and endocrine cell types. The treatment of choice is radical resection. In contrast to pancreatic neoplasms in adult patients the pancreatoblastoma has a favourable prognosis. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to the small number of patients treated as yet.