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Diagnostic Pathology
Published in: Diagnostic Pathology 1/2012

Open Access 01-12-2012 | Case Report

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

Authors: Olga L Bohn, Eric Acosta-Ponce de León, Oscar Lezama, Nina P Rios-Luna, Sergio Sánchez-Sosa, Antonio Llombart-Bosch

Published in: Diagnostic Pathology | Issue 1/2012

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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.

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Metadata
Title
Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report
Authors
Olga L Bohn
Eric Acosta-Ponce de León
Oscar Lezama
Nina P Rios-Luna
Sergio Sánchez-Sosa
Antonio Llombart-Bosch
Publication date
01-12-2012
Publisher
BioMed Central
Published in
Diagnostic Pathology / Issue 1/2012
Electronic ISSN: 1746-1596
DOI
https://doi.org/10.1186/1746-1596-7-154

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