8480/3 Mucinous carcinoma



Definitions

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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.

Mucinous adenocarcinoma of the gallbladder
These neoplasms are more common in the gallbladder than in the extrahepatic bile ducts, and are similar to those that arise at other anatomical sites. By convention, > 50% of the tumour contains extracellular mucin 1
 
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



2
 
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



. Mucinous carcinoma should be distinguished from a benign mucocele. The abundant extracellular mucin present in a mucocele may be recognized grossly in the gallbladder wall as nodules of different sizes. The mucin can extend to the serosa through the Rokitansky-Aschoff sinuses and induce a histiocytic response. The histiocytes may phagocytize mucin and be confused with signet ring cells. Immunolabelling for keratin and CEA is positive in mucinous carcinoma and negative in mucocele. Ruptured, mucin-containing, Rokitansky-Aschoff sinuses with abundant extracellular mucins having small benign glandular and papillary structures should not be confused with mucinous carcinoma 3
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Albores-Saavedra J, Galliani C, Chable-Montero F, Batich K, Henson DE (2009)
Mucin-containing Rokitansky-Aschoff sinuses with extracellular mucin deposits simulating mucinous carcinoma of the gallbladder.
Am J Surg Pathol 33: 1633-8



4
 
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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Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.

Mucoid adenocarcinoma / Colloid carcinoma of the pancreas
Colloid carcinoma is an infiltrating ductal epithelial neoplasm of the pancreas characterized by the presence, in at least 80% of the neoplasm, of large extracellular stromal mucin pools containing suspended neoplastic cells
5
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Adsay NV, Pierson C, Sarkar F, Abrams J, Weaver D, Conlon KC, Brennan MF, Klimstra DS (2001)
Colloid (mucinous noncystic) carcinoma of the pancreas.
Am J Surg Pathol 25: 26-42



. Colloid carcinomas tend to be large and well-demarcated and almost always arise in association with an intestinal-type intraductal papillary mucinous neoplasm (IPMN) 6
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Seidel G, Zahurak M, Iacobuzio-Donahue C, Sohn TA, Adsay NV, Yeo CJ, Lillemoe KD, Cameron JL, Hruban RH, Wilentz RE (2002)
Almost all infiltrating colloid carcinomas of the pancreas and periampullary region arise from in situ papillary neoplasms: a study of 39 cases.
Am J Surg Pathol 26: 56-63



. The large pools of mucin are partially lined by well-differentiated cuboidal to columnar neoplastic cells and contain clumps or strands of neoplastic cells. Some floating cells may be of the signet-ring type. The neoplastic cells of colloid carcinoma show intestinal differentiation; there is strong expression of CDX2 and MUC2, which are not significantly expressed in conventional ductal adenocarcinoma.
Two features help to distinguish benign spillage of mucin into the extraductal stroma caused by rupture of the dilated pancreatic duct occupied by an IPMN from true tissue invasion by a colloid carcinoma. Neoplastic cells ?floating? in pools of stromal mucin, and neoplastic cells in an abnormal location, such as in the perineurium, help identify the lesion as a colloid carcinoma. In contrast, benign mucin spillage will consist of mucus lakes adjacent to a pancreatic branch duct without ?floating? neoplastic cells.
Pseudomyxoma peritonei can be a rare complication of this carcinoma. Colloid carcinomas seem to have a more favourable prognosis than conventional ductal adenocarcinomas
7
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Poultsides GA, Reddy S, Cameron JL, Hruban RH, Pawlik TM, Ahuja N, Jain A, Edil BH, Iacobuzio-Donahue CA, Schulick RD, Wolfgang CL (2010)
Histopathologic basis for the favorable survival after resection of intraductal papillary mucinous neoplasm-associated invasive adenocarcinoma of the pancreas.
Ann Surg 251: 470-6



8
 
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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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.

Mucinous adenocarcinoma of the stomach is composed of malignant epithelium and extracellular mucinous pools. By convention, the tumour shows more than 50% extracellular mucin. Mucinous carcinomas may contain scattered signet-ring cells.



Thyroid gland
ICD-O-3 topography code: C73
ICD10: C73
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation. The histological hallmark of mucinous carcinoma is the presence of abundant mucoid lakes around strands or clusters of tumour cells that usually show large regular nuclei and prominent nucleoli
9
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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Mucinous carcinoma of the thyroid is very rare, with only a few cases reported
10
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Diaz-Perez R, Quiroz H, Nishiyama RH (1976)
Primary mucinous adenocarcinoma of thyroid gland.
Cancer 38: 1323-5



11
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Sobrinho-Simões M, Stenwig AE, Nesland JM, Holm R, Johannessen JV (1986)
A mucinous carcinoma of the thyroid.
Pathol Res Pract 181: 464-71



12
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Sobrinho-Simões MA, Nesland JM, Johannessen JV (1985)
A mucin-producing tumor in the thyroid gland.
Ultrastruct Pathol 9: 277-81



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

Mucinous carcinoma is characterized by clusters of generally small and uniform cells floating in large amounts of extracellular mucin.





Skin
ICD-O-3 topography code: C44
ICD10: C44
           

Cutaneous mucinous carcinoma (MC) is a rare, slow-growing, invasive adenocarcinoma characterized by large pools of mucin, which are compartmentalized by fibrous septa and contain small clusters of neoplastic epithelial cells. The neoplastic cells are cuboidal, round or oval and contain abundant cytoplasm and small nuclei with very little atypia. MC arises in the dermis and the subcutaneous fat, most frequently on the head (particularly on scalp, face and eyelids), and may extend into the subcutis or deeper. It presents as a solitary, nodular, well-circumscribed, unencapsulated lesion of tan, grey, or reddish colour. Tumour size ranges from 1 to >8 cm. On excision, the tumour appears fixed to the adjacent dermis; the cut surface is gelatinous. Tumour satellites may be observed at some distance from the main tumour. Histological differentiation between primary cutaneous MC and metastatic mucinous carcinoma to the skin may be impossible.
Mucinous adenocarcinoma develops mainly between the 5th and 7th decades of life (age range: 8-84 years) and is slightly more common in men. In contrast to most other sweat gland carcinomas and despite a destructive local growth pattern, MC generally follows an indolent course and has only a low metastatic potential. The tumour tends to persist, and multiple local recurrences are not uncommon, but death from MC is exceptional
18
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.

Mucinous adenocarcinoma of the ampulla of Vater
Approximately 5% of the ampullary carcinomas in the SEER database are mucinous adenocarcinomas
19
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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



. The diagnosis of mucinous adenocarcinoma requires that > 50% of the tumour should be composed of stromal mucin that contains small groups of malignant epithelial cells, epithelial strips or ruptured cystically dilated glands. The strips and glands are lined by columnar cells with an intestinal phenotype, whereas the cells arranged in small groups or clusters may have a signet-ring cell morphology.

Some mucinous adenocarcinomas are associated with a component of intestinal adenocarcinoma, a noninvasive papillary neoplasm or an intestinal-type adenoma. Extensive sampling may be needed to demonstrate the neoplastic cells in the mucin pools of some mucinous adenocarcinomas. Some intestinal-type adenocarcinomas contain a focal mucinous component, which is insufficient to classify the tumour as a mucinous adenocarcinoma. Since the vast majority of mucinous adenocarcinomas of the ampulla express the intestinal markers CDX2 and MUC2, these tumours are regarded as variants of intestinal-type adenocarcinomas.

The prognosis for patients with ampullary mucinous carcinomas is not better than for those with intestinal- or pancreatobiliary-type adenocarcinomas
20
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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



, in contrast to mucinous carcinomas of the breast and pancreas, and their corresponding ductal carcinomas 21
 
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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Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Mucinous adenocarcinoma is an invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation
22
 
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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Highly differentiated tumours in which most of the glands are impossible to distinguish from normal are termed minimal deviation mucinous adenocarcinomas or adenoma malignum
23
 
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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Salivary glands
ICD-O-3 topography code: C07-C08
ICD10: C07-C08
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.

Mucinous adenocarcinoma of the colon and rectum
This designation is used if > 50% of the lesion is composed of pools of extracellular mucin that contain malignant epithelium as acinar structures, layers of tumour cells, or individual tumour cells including signetring cells. The level of maturation of the epithelium determines differentiation, but many mucinous adenocarcinomas are MSI-H
24
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Greenson JK, Huang SC, Herron C, Moreno V, Bonner JD, Tomsho LP, Ben-Izhak O, Cohen HI, Trougouboff P, Bejhar J, Sova Y, Pinchev M, Rennert G, Gruber SB (2009)
Pathologic predictors of microsatellite instability in colorectal cancer.
Am J Surg Pathol 33: 126-33



25
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Leopoldo S, Lorena B, Cinzia A, Gabriella DC, Angela Luciana B, Renato C, Antonio M, Carlo S, Cristina P, Stefano C, Maurizio T, Luigi R, Cesare B (2008)
Two subtypes of mucinous adenocarcinoma of the colorectum: clinicopathological and genetic features.
Ann Surg Oncol 15: 1429-39



and therefore lowgrade. Mucinous adenocarcinomas that are microsatellite-stable (MSS) or have low levels of instability (MSI-L) behave as high-grade lesions. Carcinomas with mucinous areas of < 50% are categorized as having a mucinous component26
 
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



.



Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14
ICD10: C00-C06, C09-C10, C14
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



Fallopian tube and Uterine ligaments
ICD-O-3 topography code: C57
ICD10: C57
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



Anus and anal canal
ICD-O-3 topography code: C21
ICD10: C21
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.





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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



Corpus uteri
ICD-O-3 topography code: C54
ICD10: C54
           

Mucinous adenocarcinoma is an invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.
The prognosis is similar to that of other low-grade endometrial adenocarcinomas
and generally favourable
27
 
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



.



Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



Ovary
ICD-O-3 topography code: C56
ICD10: C56
           

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.



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

An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation.