Pancreatic adenocarcinoma and pancreatic high-grade neuroendocrine carcinoma: two sides of the moon

Pancreatic adenocarcinoma is the seventh leading cause of cancer death in the world and the most common type pf pancreatic cancer. Unfortunately, less than 20% of patients are surgically resectable and the great majority of cases are treated with palliative chemotherapy with unsatisfactory results. No targeted agents or personalized approaches have been validated in the last decades. On the other side, neuroendocrine neoplasms of the pancreas are generally considered indolent tumours. However, high-grade neuroendocrine carcinoma is a rare subtype of neuroendocrine neoplasm of the pancreas (accounting up to 10% of the neuroendocrine neoplasms of the pancreas), with particularly aggressive behaviour and poor prognosis. Even in this case, the treatment is represented by palliative chemotherapy with dismal results and no personalized therapies are available, so far. Notably, the quality of life of these patients is disappointingly low and the future perspectives of more personalized diagnostic and therapeutic strategies are scarce. In this review, we discuss relevant and current information on epidemiology, pathology, diagnosis, clinical presentation, treatment and ongoing clinical trials of these two entities, in order to illustrate the two sides of the moon.