Synonyms
Periampullary carcinoma
Definition
Ampullary carcinoma (AC) is an uncommon and heterogeneous neoplasm. AC can be broadly considered as tumors arising out of or within 1 cm of the papilla of Vater and include ampullary, pancreatic, bile duct, and duodenal carcinomas. Distinction may not be possible in advanced cases. There is a high incidence of these tumors in patients with FAP. AC can be broadly classified as primary, metastatic, or systemic. Primary cancers arise in the pancreas or other periampullary sites and can demonstrate either endocrine or non-endocrine differentiation. Ductal adenocarcinoma is by far the most common periampullary malignant tumor.
Clinical Features
Incidence
The incidence of AC was reported as 0.7 cases per 10,000 males and 0.4 cases for females between 1985 and 2005. AC represents 0.5% of all gastrointestinal malignancies.
Age
AC usually diagnosed in patients between the ages of 60 and 80 years (range 29–85 years).
Sex
ACs are more common in men than...
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References and Further Reading
Albores-Saavedra, J., Schwartz, A. M., Batich, K., & Henson, D. E. (2009). Cancers of the ampulla Vater; Demographics, morphology and survival based on 5628 cases from the SEER program. Journal of Surgical Oncology, 100, 598–605.
Albores-Saavedra, J., Hruban, R. H., Klimstra, D. S., & Zamboni, G. (2010). Invasive adenocarcinoma of the ampullary region. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (pp. 87–91). Lyon-France: IARC.
Morini, S., Perrone, G., Borzomati, D., et al. (2012). Carcinoma of the ampulla Vater. Morphological and immunophynotypical classification predicts survival. Pancreas, 42, 60–66.
Ruemmele, P., Dietmaier, W., Terracciano, L., et al. (2009). Histopathologic features and microsatellite instability of cancers of the papilla of Vater and their precursor lesions. The American Journal of Surgical Pathology, 33, 691–704.
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Savaş, B. (2017). Ampullary Carcinoma. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1429
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1429
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