8490/3 Signet ring cell carcinoma


Synonyms

Signet ring cell adenocarcinoma


Definitions

Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

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 pancreas
This is an extremely rare tumour. It is an adenocarcinoma composed almost exclusively of poorly cohesive neoplastic epithelial cells containing intracytoplasmic mucin that displaces the nuclei towards the periphery, infiltrating in an individual cell pattern 1
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Fagih M, Serra S, Chetty R (2007)
Paucicellular infiltrating ductal carcinoma of pancreas: an unusual variant.
Ann Diagn Pathol 11: 46-8



2
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Tracey KJ, O'Brien MJ, Williams LF, Klibaner M, George PK, Saravis CA, Zamcheck N (1984)
Signet ring carcinoma of the pancreas, a rare variant with very high CEA values. Immunohistologic comparison with adenocarcinoma.
Dig Dis Sci 29: 573-6



. A variable amount of extracellular mucin is usually present. The prognosis is extremely poor. A gastric or breast primary should always be excluded before making this diagnosis3
 
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



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Gallbladder and extrahepatic biliary tract
ICD-O-3 topography code: C23-C24.0
ICD10: C23-C24
           

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 gallbladder
Cells containing intracytoplasmic mucin displacing the nuclei toward the periphery predominate in this variant of adenocarcinoma. A variable amount of extracellular mucin is usually present. Lateral spread through the lamina propria is a common feature. A diffusely infiltrating linear pattern resembling linitis plastica(diffuse-type adenocarcinoma) of the stomach has been observed
4
 
Albores-Saavedra J, Henson DE, and Klimstra DS eds.
Tumors of the Gallbladder, Extrahepatic Bile Ducts, and Ampulla of Vater
Armed Forces Institute of Pathology: Washington, DC 2000



5
 
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



.



Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C18-C20
           

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 colon and rectum
This variant of adenocarcinoma is defined by the presence of > 50% of tumour cells with prominent intracytoplasmic mucin, typically with displacement and moulding of the nucleus. Signet-ring cells can occur within the pools of mucinous adenocarcinoma or in a diffusely infiltrative process with minimal extracellular mucin in a linitis-plastica pattern. Large signet-ring cells can be termed "globoid cells". Some signet ring cell carcinomas are MSI-H and are low-grade, but those that lack MSI-H are usually highly aggressive
6
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Pande R, Sunga A, Levea C, Wilding GE, Bshara W, Reid M, Fakih MG (2008)
Significance of signet-ring cells in patients with colorectal cancer.
Dis Colon Rectum 51: 50-5



. Carcinomas with signet-ring cell areas of < 50% are categorized as adenocarcinoma with a signet-ring cell component 7
 
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



.



Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Signet ring cell carcinoma is 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. In pure form, primary signet-ring cell adenocarcinoma is rare. Signet-ring cells are more commonly found focally in poorly differentiated mucinous adenocarcinomas and adenosquamous carcinomas
8
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Region of the ampulla of Vater
ICD-O-3 topography code: C24.1
ICD10: C24.1
           

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
9
Click to access Pubmed
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 10
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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



11
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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



12
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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 13
 
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



.



Breast
ICD-O-3 topography code: C50
ICD10: C50
           

Signet ring cell carcinoma is 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
14
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



.



Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

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.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

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.



Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

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.



Bronchus and lung
ICD-O-3 topography code: C34
ICD10: C34
           

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.