| Undifferentiated carcinoma with osteoclastic giant cells (UCOCGC) of the pancreas associated with the familial atypical multiple mole melanoma syndrome (FAMMM). | |
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MedLine Citation:
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PMID: 18813118 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The familial atypical multiple mole melanoma (FAMMM) syndrome is caused by a germline mutation of p16. More than 90% of the sporadic pancreatic carcinomas contain genetic alterations that inactivate p16. Patients with the FAMMM syndrome have an increased risk of developing pancreatic cancer. Ductal adenocarcinoma is the most common cancer of the pancreas and the one encountered in patients with FAMMM syndrome. Undifferentiated carcinoma with osteoclastic giant cells, also referred to as UCOCGC of the pancreas, is a rare variant of pancreatic cancer. An UCOCGC of the pancreas associated with FAMMM syndrome is described in this report. Molecular analysis confirmed a germline p16-Leiden deletion in the UCOCGC, accompanied by somatic loss of heterozygosity of the second p16 allele, and absence of p16 protein expression in the neoplastic cells. It is the first case reported and it provides additional evidence that UCOCGC can be considered as a variant of conventional ductal adenocarcinoma of the pancreas. |
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Authors:
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Jan-Bart M Koorstra; Anirban Maitra; Folkert H M Morsink; Paul Drillenburg; Fiebo J W ten Kate; Ralph H Hruban; Johan A Offerhaus |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: The American journal of surgical pathology Volume: 32 ISSN: 1532-0979 ISO Abbreviation: Am. J. Surg. Pathol. Publication Date: 2008 Dec |
Date Detail:
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Created Date: 2008-12-04 Completed Date: 2009-01-23 Revised Date: 2011-01-14 |
Medline Journal Info:
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Nlm Unique ID: 7707904 Medline TA: Am J Surg Pathol Country: United States |
Other Details:
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Languages: eng Pagination: 1905-9 Citation Subset: IM |
Affiliation:
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Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Calcinosis / pathology Carcinoma, Pancreatic Ductal / complications*, genetics, pathology Cyclin-Dependent Kinase Inhibitor p16 / biosynthesis, genetics Dysplastic Nevus Syndrome / complications*, genetics, pathology Gene Deletion Genes, p16 Giant Cells / pathology* Humans Immunohistochemistry Loss of Heterozygosity Male Osteoclasts / pathology Pancreatic Neoplasms / complications*, genetics, pathology Pedigree Syndrome |
| Grant Support | |
ID/Acronym/Agency:
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CA62924/CA/NCI NIH HHS; P50 CA062924-150016/CA/NCI NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Cyclin-Dependent Kinase Inhibitor p16 |
| Comments/Corrections | |
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