What is the definition of Familial Pancreatic Cancer?

Familial pancreatic cancer (FPC) is the occurrence of pancreatic cancer in two or more first-degree relatives (parent and child, or two siblings). It is sometimes referred to as FPC only when there is not a known hereditary cancer syndrome in an affected family. In familial cases, pancreatic cancer often occurs before age 50 (earlier than other forms of pancreatic cancer). In 60% of cases it occurs within the head of the pancreas. Symptoms of pancreatic cancer are generally non-specific and may include pain in the upper abdomen that radiates to the back; loss of appetite; significant weight loss; and jaundice due to bile duct obstruction. Pancreatic cancer often goes undetected until the advanced stages of the disease, and rapid tumor growth and metastasis are common.

A specific genetic cause of FPC (in the absence of a known syndrome) has not been identified. Familial cases of pancreatic cancer due to hereditary conditions may be caused by mutations in any of several genes. Individuals who carry a mutation in one of these genes are often at an increased risk of other types of cancers as well. Currently, only 10-20% of families with FPC will have a mutation identified by genetic testing. Hereditary cancer syndromes and other inherited conditions associated with pancreatic cancer include:
  • BRCA1 hereditary breast and ovarian cancer syndrome
  • BRCA2 hereditary breast and ovarian cancer syndrome
  • Peutz-Jeghers syndrome
  • FAMMM syndrome
  • Lynch syndrome
  • Hereditary pancreatitis
In many cases, inheritance of FPC is autosomal dominant; in some cases, is may be multifactorial. It is important to note that pancreatic cancer itself is not inherited, but a genetic predisposition to developing cancer is inherited.

Treatment may include surgical resection, chemotherapy, and/or radiotherapy. Resection is the only potential cure, but most patients have advanced, non-resectable tumors by the time of diagnosis.

What are the alternative names for Familial Pancreatic Cancer?

  • Hereditary pancreatic carcinoma
  • Familial pancreatic carcinoma
  • Hereditary pancreatic cancer
  • Condition: Metastatic Neuroendocrine Tumors
  • Journal: World journal of gastroenterology
  • Treatment Used: 177Lu Peptide Receptor Radionuclide Therapy
  • Number of Patients: 36
  • Published —
The study researched the outcomes of 177Lu peptide receptor radionuclide therapy in patients with metastatic neuroendocrine tumors.
  • Condition: Metastatic Adult Pancreatoblastoma
  • Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
  • Treatment Used: Oxaliplatin Multimodal Treatment
  • Number of Patients: 4
  • Published —
This case report describes using various multimodal treatments to treat patients with metastatic adult pancreatoblastoma.
Clinical Trial
  • Status: Not yet recruiting
  • Intervention Type: Other, Diagnostic Test, Drug
  • Participants: 100000
  • Start Date: April 5, 2021
The Registry of Oncology Outcomes Associated With Testing and Treatment (ROOT)