Mixed-type pancreatoblastoma. Report of one case [Pancreatoblastoma di tipo misto. Presentazione di un caso clinico]

Pancreatoblastoma is a very rare tumor of the pancreas, affecting children in the first decade of life. Ultrasound and CT scan may be useful but pre-operative diagnosis is often quite difficult. High level of seric ?-fetoprotein could be suggestive of pancreatoblastoma. The definitive diagnosis is however made by histologists when an organoid structure, well encapsulated, consisting of acinar cells with zymogen-like granules and squamoid corpuscle is demonstrated, sometimes associated with mesenchymal tissue (mixed-type pancreatoblastoma). Immunohistochemical studies may help in differentiating pancreatoblastoma from papillary cystic tumors, acinar cell carcinomas and endocrine pancreatic tumors of the pancreas. We report a case of mixed-type pancreatoblastoma in a 12-year-old female patient, located in the head of the pancreas. The treatment has been surgical, consisting of a duodeno-cephalo-pancreasectomy. Neither radiotherapy nor chemotherapy has been performed. The patient is alive, without recurrence, four months after the operation. Diagnostic, therapeutic and prognostic aspects are discussed.