pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma a case report肺动脉肉瘤与血管肉瘤表型模仿多形性恶性纤维组织细胞瘤病例报告.pdfVIP

Bohn et al. Diagnostic Pathology 2012, 7:154 /content/7/1/154 CASE REPORT Open Access Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report 1,5* 2 3 1 1 Olga L Bohn , Eric Acosta-Ponce de León , Oscar Lezama , Nina P Rios-Luna , Sergio Sánchez-Sosa and Antonio Llombart-Bosch4 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. Keywords: Pulmonary artery, Sarcoma, Angiosarcoma, Immunohistochemistry Background Case presentation Primar