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Pancreatic ductal adenocarcinoma (PDAC) is a disease marked by high rates of mortality; it is mostly incurable at the time of diagnosis. Only about 7% of patients survive 5 years after diagnosis. Diagnosis at a late stage and rapid progression with minimal response to available treatments are the main reasons for this poor outcome. It is crucial to identify individuals at high risk of developing PDAC so preventive and early detection measures can be employed. Approximately 10% to 15% of PDAC cases have a hereditary or familial basis. In the majority of PDAC cases, no main causative gene has been identified, but several known germline pathogenic mutations have been shown to be related to an increased risk of this cancer. The presence of 2 or more patients with pancreatic cancer within the circle of first-degree relatives, without the presence of a causative germline mutation, is defined as familial pancreatic cancer; this accounts for 4% to 10% of PDAC. Based on the growing evidence supporting the benefit of germline genetic testing in patients with PDAC, both the American Society of Clinical Oncology and the National Comprehensive Cancer Network recently updated their guidelines to include recommendations around genetic testing for patients with pancreatic cancer. However, there is no general consensus on the group of patients and individuals who should be studied and screened. We present a demonstrative case and review the available data on hereditary and familial PDAC. KEYWORDS: pancreatic ductal adenocarcinoma, hereditary, familial, mutation, screening, prevention, Introduction Pancreatic ductal adenocarcinoma (PDAC) has one of the worst prognoses among major cancers. In 2020 in the United States, it is expected to be the third-leading cause of cancer [...]

Biliary cancer is a highly aggressive malignancy arising from the biliary tree, with its incidence increasing steadily on a global level. Most biliary cancers are diagnosed in the advanced and metastatic stages due to the paucity of signs and symptoms in the early presentation. Only about one-third of the patients can be treated with curative intent with an overall median survival of less than 24 months for all-comers from the time of diagnosis. This fact and the poor results of the currently available local and systemic therapies, are responsible for the disappointing outcome of biliary cancer patients. There is an unmet need for novel therapeutic approaches. Surgery, the backbone of curative treatments for biliary cancer, is effective in early, completely-resectable stages or in combination with neoadjuvant or adjuvant chemotherapy and/or radiation therapy for locally advanced stages. Systemic therapies in unresectable and recurrent cases are associated with poor outcomes. The introduction of next-generation sequencing technologies has opened new horizons for a better understanding of the molecular basis of this cancer with potential identification and evaluation of new treatment options., Introduction Biliary cancers are a heterogeneous group of tumors that originate from the epithelial lining cells of the small ducts within the liver and the main ducts of the biliary [...]