8470/3 Pseudomucinous cystadenocarcinoma, NOS



Definitions

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

An invasive adenocarcinoma characterized by cystic changes and the presence of malignant glandular cells which contain intracytoplasmic mucin.

Mucinous cystic neoplasms (MCN) with an associated invasive carcinoma of the pancreas
Mucinous cystic neoplasms (MCN) are categorized as:
> MCN with 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.

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 167]1
 
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 2
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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. The prognosis for patients with an MCN with an associated invasive carcinoma depends on the extent(depth) of the invasive component, tumour stage (lymph-node and distant metastasis), and resectability 3
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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



. The 2-year survival rate for patients with a surgically resected MCN with an associated invasive carcinoma is about 67%, and the 5-year survival rate is about 50% 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
 
Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



6
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Le Borgne J, de Calan L, Partensky C (1999)
Cystadenomas and cystadenocarcinomas of the pancreas: a multiinstitutional retrospective study of 398 cases. French Surgical Association.
Ann Surg 230: 152-61



7
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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



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



.



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

Mucinous cystadenocarcinoma and columnar cell mucinous carcinoma of the breast are rare invasive carcinomas characterized by large columnar cells with bland, basal nuclei and abundant intracytoplasmic mucin. The tumours are cystic and vary in size from 0.8 to 19 cm.
The two variants are distinguished according to the appearance of the intracytoplasmic mucin: tumours with cystic mucin are classified as mucinous cystadenocarcinoma, those with solid mucin as columnar cell mucinous carcinoma.
The cystic variant is histologically characterized by numerous cysts, focal atypia, loss of polarity, eosinophilic cellular transformation and invasion of the surrounding stroma.
The columnar cell variant consists of compact to loose aggregations of round and convoluted glands lined by a single layer of columnar mucinous epithelium.
The very few reported cases of mucinous cystadenocarcinoma and columnar cell mucinous carcinoma occurred in women 49 to 67 years of age
9
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Koenig C, Tavassoli FA (1998)
Mucinous cystadenocarcinoma of the breast.
Am J Surg Pathol 22: 698-703



. Recurrence or metastasis has not been observed 10
 
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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Testis/Male genital organs
ICD-O-3 topography code: C62-C63
ICD10: C62-C63
           

An invasive adenocarcinoma characterized by cystic changes and the presence of malignant glandular cells which contain intracytoplasmic mucin.



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

An invasive adenocarcinoma characterized by cystic changes and the presence of malignant glandular cells which contain intracytoplasmic mucin.