Gaumann, Andreas and Bode-Lesniewska, B. and Zimmermann, D. R. and Fanburg-Smith, J. C. and Kirkpatrick, C. J. and Hofstaedter, F. and Woenckhaus, M. and Stoehr, R. and Obermann, E. C. and Dietmaier, W. and Hartmann, A. (2008) Exploration of the APC/beta-catenin (WNT) pathway and a histologic classification system for pulmonary artery intimal sarcoma. A study of 18 cases. VIRCHOWS ARCHIV, 453 (5). pp. 473-484. ISSN 0945-6317,
Full text not available from this repository. (Request a copy)Abstract
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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | POLYPOSIS-COLI GENE; CYCLIN D1; EPITHELIOID ANGIOSARCOMA; AGGRESSIVE FIBROMATOSIS; EXPRESSION; MUTATIONS; TUMORS; OVEREXPRESSION; PROTEIN; CELLS; Pulmonary artery sarcoma; APC; beta-catenin; Cyclin D1; Cyclin A; LOH |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Pathologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 21 Oct 2020 09:34 |
| Last Modified: | 21 Oct 2020 09:34 |
| URI: | https://pred.uni-regensburg.de/id/eprint/30152 |
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