8470/2 Mucinous cystadenocarcinoma, non-invasive



Definitions

Pancreas
ICD-O-3 topography code: C25

A non-invasive malignant cystic epithelial neoplasm composed of cells which contain intracytoplasmic mucin.

Mucinous cystic neoplasms (MCN) with high-grade of dysplasia of the pancreas
Mucinous cystic neoplasms (MCN) are categorized as:
> MCN with low low-or intermediate-grade of dysplasia, previously called mucinous cystadenoma
> MCN with high grade of dysplasia, previously called mucinous cystadenocarcinoma, non-invasive
> MCN with an associated invasive carcinoma if there is a component of invasive carcinoma.

In MCNs with high grade of dysplasia, there is cellular stratification, severe dysplasia, and high mitotic activity present.

MCNs of the pancreas are cyst-forming epithelial neoplasm that usually does not communicate with the pancreatic ductal system, and is composed of columnar, mucin-producing epithelium associated with ovarian-type subepithelial stroma 1
 
Hamilton SR, Aaltonen LA (eds.)
WHO Classification of Tumours of the Digestive System.
IARC
Lyon 2000



2
 
Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



. This neoplasm occurs almost exclusively in women. MCNs are relatively rare, accounting for about 8% of surgically resected cystic lesions of the pancreas 3
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Kosmahl M, Pauser U, Peters K, Sipos B, Lüttges J, Kremer B, Klöppel G (2004)
Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: a review of 418 cases and a classification proposal.
Virchows Arch 445: 168-78



. A MCN should be suspected whenever a cystic lesion is seen by endoscopic ultrasonography (EUS), computed tomography (CT) or magnetic resonance imaging (MRI) in the pancreatic body?tail of a young or middle-aged woman, especially in the absence of a history of pancreatitis. MCNs have two distinct components ? an epithelial lining and an underlying ovariantype stromal component. The possibility that the stromal component of MCNs is derived from ovarian primordium is supported by morphology, tendency to undergo luteinization, presence of hilar-like cells, and immunophenotypic sex cord?stromal differentiation. Surgical resection is curative for almost all patients with a noninvasive MCN 4
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Crippa S, Salvia R, Warshaw AL, Domínguez I, Bassi C, Falconi M, Thayer SP, Zamboni G, Lauwers GY, Mino-Kenudson M, Capelli P, Pederzoli P, Castillo CF (2008)
Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients.
Ann Surg 247: 571-9



5
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Sarr MG, Carpenter HA, Prabhakar LP, Orchard TF, Hughes S, van Heerden JA, DiMagno EP (2000)
Clinical and pathologic correlation of 84 mucinous cystic neoplasms of the pancreas: can one reliably differentiate benign from malignant (or premalignant) neoplasms?
Ann Surg 231: 205-12



6
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Wilentz RE, Albores-Saavedra J, Zahurak M, Talamini MA, Yeo CJ, Cameron JL, Hruban RH (1999)
Pathologic examination accurately predicts prognosis in mucinous cystic neoplasms of the pancreas.
Am J Surg Pathol 23: 1320-7



7
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Zamboni G, Scarpa A, Bogina G, Iacono C, Bassi C, Talamini G, Sessa F, Capella C, Solcia E, Rickaert F, Mariuzzi GM, Klöppel G (1999)
Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors.
Am J Surg Pathol 23: 410-22



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



.





Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22

A non-invasive malignant cystic epithelial neoplasm composed of cells which contain intracytoplasmic mucin.

In the liver, this cyst-forming epithelial neoplasm usually shows no communication with the bile ducts, composed of cuboidal to columnar, variably mucin-producing epithelium, associated with ovarian-type subepithelial stroma. Noninvasive mucinous cystic neoplasms (MCN) are categorized on the basis of the highest degree of cytoarchitectural atypia into MCN with low or intermediated grade dysplasia, and MCN with high-grade dysplasia. If there is a component of invasive carcinoma, the lesion should be designated as MCN with an associated invasive carcinoma. These neoplasms are rare and occur almost exclusively in women. MCNs occur principally in the liver and occasionally in the extrahepatic biliary system (including the gallbladder)
9
 
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



. In high-grade tumours, there is cellular stratification, severe dysplasia, and high mitotic activity present. These tumours must be differentiated from intraductal papillary neoplasia (IPN), hepatic microcystic serous cystadenoma of pancreatic type, biliary fibroadenoma, endometriosis and bile duct and peribiliary cysts. The prognosis for patients with a noninvasive biliary MCN is excellent if complete resection is possible 10
 
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



.





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

A non-invasive malignant cystic epithelial neoplasm composed of cells which contain intracytoplasmic mucin.

Mucinous cystic neoplasms (MCNs) of the gallbladder and extrahepatic bile ducts resemble their intrahepatic counterparts. They are seen predominantly in adult females and are usually symptomatic. Some MCNs measure up to 20 cm in diameter and cause obstructive jaundice or cholecystitis-like symptoms. More common in the extrahepatic bile ducts than in the gallbladder, MCNs are multiloculated neoplasms that contain mucinous or serous fluid and are lined by columnar epithelium reminiscent of bile duct or foveolar gastric epithelium
11
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Devaney K, Goodman ZD, Ishak KG (1994)
Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients.
Am J Surg Pathol 18: 1078-91



. Neuroendocrine cells are occasionally present. By definition, the cellular subepithelial stroma resembles ovarian stroma and is immunoreactive for estrogen and progesterone receptors. The stroma is also variably fibrotic. Invasive carcinomas that arise in association with MCNs should be designated MCN with an associated invasive carcinoma12
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Devaney K, Goodman ZD, Ishak KG (1994)
Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients.
Am J Surg Pathol 18: 1078-91



and clearly described in terms of grade and extent; staging is only required for the invasive component 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



.

For more details please see MCNs of the liver and intrahepatic bile ducts
http://www.pubcan.org/icdotopo.php?id=5873