Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report
1 Department of Pathology, Christus Mugerza UPAEP University Hospital, Puebla, 72000, Mexico
2 Department of Surgical Oncology, Hospital Angeles, 5 Poniente 715, Puebla, Mexico
3 Department of Radiology, Hospital Angeles, Puebla, México
4 Department of Pathology, Valencia University, Valencia, Spain
5 Current affiliation: Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Citation and License
Diagnostic Pathology 2012, 7:154 doi:10.1186/1746-1596-7-154Published: 7 November 2012
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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