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 slideThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045.
Highlights
Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall
Angiosarcomas are malignant tumors that arise from the endothelial lining of the blood vessels [1]
Primary sarcomas arising from major blood vessels are Monoclonal mouse V9
Summary
Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall (mural or intraluminal ( known as “intimal”) or by histologic type. Chest X-rays and a computed tomography (CT) scan showed two right perihilar lung masses, each measuring 3.5 and 2.5 cm in greater diameter, adjacent to the main right pulmonary artery (Figure 1). Cut sections showed a right pulmonary artery thickening and a necrotic and hemorrhagic tumor, filling and near occluding the vascular lumen (Figure 2). Radiological and histopathological findings, the tumor was diagnosed as angiosarcoma arising on the pulmonary artery. The patient was disease free 18 months and the tumor recurred (additional biopsies were taken showing similar histopathological findings). She developed metastatic disease to liver and contralateral lung and died 29 months after the recurrence. Primary sarcomas arising from major blood vessels (aorta, pulmonary artery, inferior vena cava) are
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