Source:http://linkedlifedata.com/resource/pubmed/id/18807072
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
5
|
| pubmed:dateCreated |
2008-10-21
|
| pubmed:abstractText |
APC, a tumor suppressor gene in the Wnt pathway, stabilizes beta-catenin and controls cell growth. Mutation of APC or beta-catenin leads to nuclear accumulation of beta-catenin and transcription of cyclin D1/cyclin A. Pulmonary artery sarcoma (PAS) were studied by morphologic, immunohistochemical, and molecular genetic methods of the Wnt pathway. Eighteen cases were included: mean age 52 years, primary intraluminal location with typical clinical presentation. PAS were classified as epithelioid (n = 4) or malignant fibrous histiocytoma (MFH; spindled/pleomorphic, n = 4), myxofibrosarcoma (n = 8), and one each hemangiopericytoma-like or malignant inflammatory myofibroblastic tumor-like. The tumor cells demonstrated vimentin, focal actins, and rare focal desmin positivity. All but one were grade 2 or 3 by FNCLCC grading. Alteration in chromosome 5q21 (APC) was found in 4/14 PAS by LOH, mostly epithelioid-type; an MFH-type case demonstrated microsatellite instability (MSI) and nuclear beta-catenin. Cyclin D1 was expressed in seven tumors, all myxofibrosarcoma-type. No mutations were detected in APC or beta-catenin. In summary, PAS are predominantly intermediate grade myxofibrosarcoma in middle-aged males, and fatal in two-thirds of patients. Despite myofibroblastic phenotype, APC/beta-catenin pathway changes are rare. Cyclin D1, only expressed in the myxofibrosarcoma-type, is likely transcribed via factors other than beta-catenin.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Nov
|
| pubmed:issn |
0945-6317
|
| pubmed:author |
pubmed-author:Bode-LesniewskaBB,
pubmed-author:DietmaierWW,
pubmed-author:Fanburg-SmithJ CJC,
pubmed-author:GaumannAA,
pubmed-author:HartmannAA,
pubmed-author:HofstädterFF,
pubmed-author:KirkpatrickC JCJ,
pubmed-author:ObermannE CEC,
pubmed-author:StoehrRR,
pubmed-author:WoenckhausMM,
pubmed-author:ZimmermannD RDR
|
| pubmed:issnType |
Print
|
| pubmed:volume |
453
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
473-84
|
| pubmed:meshHeading |
pubmed-meshheading:18807072-Adenomatous Polyposis Coli Protein,
pubmed-meshheading:18807072-Adult,
pubmed-meshheading:18807072-Aged,
pubmed-meshheading:18807072-Cyclin A,
pubmed-meshheading:18807072-Cyclin D1,
pubmed-meshheading:18807072-Female,
pubmed-meshheading:18807072-Humans,
pubmed-meshheading:18807072-Loss of Heterozygosity,
pubmed-meshheading:18807072-Male,
pubmed-meshheading:18807072-Middle Aged,
pubmed-meshheading:18807072-Pulmonary Artery,
pubmed-meshheading:18807072-Retrospective Studies,
pubmed-meshheading:18807072-Sarcoma,
pubmed-meshheading:18807072-Sequence Analysis, DNA,
pubmed-meshheading:18807072-Signal Transduction,
pubmed-meshheading:18807072-Tunica Intima,
pubmed-meshheading:18807072-Vascular Neoplasms,
pubmed-meshheading:18807072-beta Catenin
|
| pubmed:year |
2008
|
| pubmed:articleTitle |
Exploration of the APC/beta-catenin (WNT) pathway and a histologic classification system for pulmonary artery intimal sarcoma. A study of 18 cases.
|
| pubmed:affiliation |
Institute of Pathology, University of Regensburg, Franz-Josef Strauss Allee 11, D-93042, Regensburg, Germany. andreas.gaumann@klinik.uni-regensburg.de
|
| pubmed:publicationType |
Journal Article
|