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Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report

Authors :
Bohn Olga L
de León Eric
Lezama Oscar
Rios-Luna Nina P
Sánchez-Sosa Sergio
Llombart-Bosch Antonio
Source :
Diagnostic Pathology, Vol 7, Iss 1, p 154 (2012)
Publication Year :
2012
Publisher :
BMC, 2012.

Abstract

Abstract Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome. Virtual slide The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045.

Details

Language :
English
ISSN :
17461596
Volume :
7
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Diagnostic Pathology
Publication Type :
Academic Journal
Accession number :
edsdoj.8f588c9283034f25bd83036a149b58c6
Document Type :
article
Full Text :
https://doi.org/10.1186/1746-1596-7-154