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WHO Classification of Tumours
Signet ring cell carcinoma
Region of the ampulla of Vater


A usually aggressive, poorly differentiated invasive adenocarcinoma characterized by the presence of malignant glandular cells in which the nucleus is pressed to one side by the presence of intracytoplasmic mucus.

Signet ring cell carcinoma of the ampulla of Vater
Signet ring cell carcinoma is a highly malignant neoplasm that represents 2% of all carcinomas of the ampulla

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Albores-Saavedra J, Schwartz AM, Batich K, Henson DE (2009)
Cancers of the ampulla of vater: demographics, morphology, and survival based on 5,625 cases from the SEER program.
J Surg Oncol 100: 598-605

. It consists predominantly of epithelial cells with cytoplasmic mucin that pushes the nuclei towards the periphery of the cells
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Akatsu T, Aiura K, Takahashi S, Kameyama K, Kitajima M, Kitagawa Y (2007)
Signet-ring cell carcinoma of the ampulla of Vater: report of a case.
Surg Today 37: 1110-4

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Bloomston M, Walker M, Frankel WL (2006)
Radical resection in signet ring carcinoma of the ampulla of Vater: report of an 11-year survivor.
Am Surg 72: 193-5

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Eriguchi N, Aoyagi S, Jimi A (2003)
Signet-ring cell carcinoma of the ampulla of Vater: report of a case.
Surg Today 33: 467-9

. Some neoplastic cells, however, contain little or no cytoplasmic mucin and appear undifferentiated. A minor component of tubular adenocarcinoma and clusters of neuroendocrine cells have been identified in a few signet ring cell carcinomas. Although signet ring cells can be found in mucinous adenocarcinomas, the diagnosis of signet ring cell carcinoma is reserved for those cases with the characteristic diffuse pattern of infiltration, usually without significant extracellular mucin accumulation. Tumours with mild atypia, few mitotic figures and endocrine cells can be confused with goblet cell carcinoid tumours. The infiltrating growth pattern, lack of a nesting pattern, and the mildly atypical neoplastic signet-ring cells favour a diagnosis of signet ring cell carcinoma
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010