Exocrine pancreatic neoplasms represent up to 95PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 510textless 5 with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 850 which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
- Pancreatic acinar cell cancer
- Pancreatic adenosquamous cancer
- Pseudopapillary pancreatic cancer
- Rare pancreatic cancers
- Undifferentiated pancreatic cancer