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WHO Classification of Tumours
Undifferentiated carcinoma with osteoclast-like cells
Pancreas


Definition

A usually aggressive malignant epithelial neoplasm composed of giant cells resembling osteoclasts and atypical cells that do not display glandular or squamous differentiation.

Undifferentiated carcinoma with osteoclast-like cells of the pancreas
This is a rare neoplasm composed of round to spindle-shaped, highly pleomorphic neoplastic mononuclear cells and large, non-neoplastic multinucleated, histiocytic giant cells. The latter usually contain > 20 uniform and small nuclei, and are often found in areas adjacent to haemorrhage or necrosis. In most cases there is an associated in situ or invasive adenocarcinoma or an associated mucinous cystic neoplasm (MCN)

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Molberg KH, Heffess C, Delgado R, Albores-Saavedra J (1998)
Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas and periampullary region.
Cancer 82: 1279-87




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Westra WH, Sturm P, Drillenburg P, Choti MA, Klimstra DS, Albores-Saavedra J, Montag A, Offerhaus GJ, Hruban RH (1998)
K-ras oncogene mutations in osteoclast-like giant cell tumors of the pancreas and liver: genetic evidence to support origin from the duct epithelium.
Am J Surg Pathol 22: 1247-54




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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 osteoclast-like giant cells can be phagocytically active and may contain haemosiderin. Some of the mononuclear cells are atypical, while others can be histiocyte-like
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Sakai Y, Kupelioglu AA, Yanagisawa A, Yamaguchi K, Hidaka E, Matsuya S, Ohbuchi T, Tada Y, Saisho H, Kato Y (2000)
Origin of giant cells in osteoclast-like giant cell tumors of the pancreas.
Hum Pathol 31: 1223-9



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Immunohistochemically, most of the neoplastic mononuclear cells express vimentin, some express keratin, and some label with antibodies to p53. On the other hand, osteoclast-like giant cells, and a subset of the mononuclear cells, express CD68, vimentin, and leukocyte common antigen, but are negative for keratin and do not label with antibodies to p53

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Hoorens A, Prenzel K, Lemoine NR, Klöppel G (1998)
Undifferentiated carcinoma of the pancreas: analysis of intermediate filament profile and Ki-ras mutations provides evidence of a ductal origin.
J Pathol 185: 53-60




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Lukás Z, Dvorák K, Kroupová I, Valásková I, Habanec B (2006)
Immunohistochemical and genetic analysis of osteoclastic giant cell tumor of the pancreas.
Pancreas 32: 325-9




Click to access Pubmed
Molberg KH, Heffess C, Delgado R, Albores-Saavedra J (1998)
Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas and periampullary region.
Cancer 82: 1279-87




Click to access Pubmed
Westra WH, Sturm P, Drillenburg P, Choti MA, Klimstra DS, Albores-Saavedra J, Montag A, Offerhaus GJ, Hruban RH (1998)
K-ras oncogene mutations in osteoclast-like giant cell tumors of the pancreas and liver: genetic evidence to support origin from the duct epithelium.
Am J Surg Pathol 22: 1247-54



. These immunolabelling studies, when combined with molecular analyses, have shown that the pleomorphic mononuclear cells are the neoplastic cells, while the osteoclast-like giant cells are reactive non-neoplastic cells
Click to access Pubmed
Westra WH, Sturm P, Drillenburg P, Choti MA, Klimstra DS, Albores-Saavedra J, Montag A, Offerhaus GJ, Hruban RH (1998)
K-ras oncogene mutations in osteoclast-like giant cell tumors of the pancreas and liver: genetic evidence to support origin from the duct epithelium.
Am J Surg Pathol 22: 1247-54



.
The mean age of patients with an undifferentiated carcinoma with osteoclast-like giant cells is 62 years, but there is a wide range (32–93 years)

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




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Nojima T, Nakamura F, Ishikura M, Inoue K, Nagashima K, Kato H (1993)
Pleomorphic carcinoma of the pancreas with osteoclast-like giant cells.
Int J Pancreatol 14: 275-81



. Recent studies have shown that the prognosis is poor and mean survival is only 12 months
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Cubilla AL, Fitzgerald PJ (eds)
Tumours of the Exocrine Pancreas.
Armed Forces Institute of Pathology: Washington, DC 1984




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



. Interestingly, in situ as well as early-stage undifferentiated carcinomas with osteoclast-like giant cells have also been reported
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Bergmann F, Esposito I, Michalski CW, Herpel E, Friess H, Schirmacher P (2007)
Early undifferentiated pancreatic carcinoma with osteoclastlike giant cells: direct evidence for ductal evolution.
Am J Surg Pathol 31: 1919-25




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Tezuka K, Yamakawa M, Jingu A, Ikeda Y, Kimura W (2006)
An unusual case of undifferentiated carcinoma in situ with osteoclast-like giant cells of the pancreas.
Pancreas 33: 304-10



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