Familial pancreatic carcinoma.
Summary of "Familial pancreatic carcinoma."
Less than 10% of pancreatic ductal adenocarcinomas are based on a hereditary syndrome. In contrast a positive family history for pancreatic cancer raises the individual risk for the development of pancreatic and extrapancreatic malignancies.While 70% of hereditary pancreatic carcinomas can be attributed to the familiar pancreatic cancer syndrome 30% are caused by other hereditary syndromes, e.g., Peutz-Jeghers syndrome or familial adenomatous polyposis. Furthermore, genetically determined pancreatitis (e.g., hereditary pancreatitis or cystic fibrosis) can lead to pancreatic cancer.Up to now conclusive data for routine screening of high risk patients are not available which is due in part to the difficult identification of high risk patients and the problematic classification of detected pancreatic lesions. Therefore, high risk patients should be included in controlled clinical trials for screening. Small pancreatic lesions are not clear indications for surgical resection as false positive results can hamper a clear diagnosis and prophylactic pancreatectomy is not recommended. In the case of a histologically proven carcinoma a prophylactic extension of resection might be reasonable. Prevention of familial pancreatic cancer can be achieved through nicotine abstinence.
Klinik und Poliklinik für Viszeral-, Thorax- und Gefäßchirurgie, Universitätsklinikum Carl Gustav Carus Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland.
This article was published in the following journal.
Name: Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21487813
- DOI: http://dx.doi.org/10.1007/s00104-011-2084-x
Medical and Biotech [MESH] Definitions
Carcinoma, Pancreatic Ductal
Carcinoma that arises from the PANCREATIC DUCTS. It accounts for the majority of cancers derived from the PANCREAS.
Carcinoma, Islet Cell
A primary malignant neoplasm of the pancreatic ISLET CELLS. Usually it involves the non-INSULIN-producing cell types, the PANCREATIC ALPHA CELLS and the pancreatic delta cells (SOMATOSTATIN-SECRETING CELLS) in GLUCAGONOMA and SOMATOSTATINOMA, respectively.
A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)
Von Hippel-lindau Disease
An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include HEMANGIOBLASTOMA in the RETINA; CEREBELLUM; and SPINAL CORD; PHEOCHROMOCYTOMA; pancreatic tumors; and renal cell carcinoma (see CARCINOMA, RENAL CELL). Common clinical signs include HYPERTENSION and neurological dysfunctions.
A 36-amino acid pancreatic hormone that is secreted mainly by endocrine cells found at the periphery of the ISLETS OF LANGERHANS and adjacent to cells containing SOMATOSTATIN and GLUCAGON. Pancreatic polypeptide (PP), when administered peripherally, can suppress gastric secretion, gastric emptying, pancreatic enzyme secretion, and appetite. A lack of pancreatic polypeptide (PP) has been associated with OBESITY in rats and mice.
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