Angiosarcoma is an extremely aggressive tumor with a high rate of mortality. It can arise in any tissue of the body and is most commonly found in the skin and soft tissue[1]. Pulmonary angiosarcoma is usually secondary to a primary location of the body and presents as both solitary and multiple nodules. Primary pulmonary angiosarcoma is a rare disease with less than 20 cases reported in the English literature. In our report we present a case of metastatic primary pulmonary angiosarcoma involving the most ( > 100) pulmonary nodules known to date. Novel treatment strategy using an anti-angiogenic inhibitor was used to treat this disease for the first time to our knowledge;however, it was unsuccessful in controlling disease progression. This report reviews the literature of this rare and devastating disease.
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