Could the burden of pancreatic cancer originate in childhood?

Abstract

The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Many pediatric cancers may present pancreatic metastases, such as renal cell carcinoma, which may evolve with pancreatic metastatic disease even after two or more decades. Several childhood diseases may create a predisposition for the development of pancreatic cancer during adulthood; hence, there is a need for extensive screening strategies and complex programs to facilitate the transition from pediatric to adult healthcare. Nevertheless, genetic studies highlight the fact the specific gene mutations and family aggregations may be correlated with a special predisposition towards pancreatic cancer. This review aims to report the main pancreatic cancers diagnosed during childhood, the most important childhood diseases predisposing to the development of pancreatic malignancies, and the gene mutations associates with pancreatic malignant tumors.

Keywords: Pancreatic cancer; Pancreatic metastasis; Childhood; Adult; Screening; Transition

Core Tip: Pancreatic malignant tumors are rarely found during childhood, their prognosis being linked to the type of tumor and its capacity to evolve towards metastasis. Also, there are types of cancer diagnosed in pediatric patients which may present with pancreatic metastasis later, during adulthood. Various conditions, when diagnosed during childhood, may be associated with the later onset of pancreatic cancer. Also, several genetic mutations have been linked to the development of pancreatic malignancies. We discuss here the above-mentioned topics in the context of a comprehensive literature review.