Diffuse Primary Angiosarcoma of the Pleura: A Case Report and Review of the Literature

Abstract

Primary pleural angiosarcoma is an extremely rare tumor. We report the case of a patient who presented with recurrent massive bilateral hemothoraxes. Although thoracoscopy was performed, biopsy samples of the pleura were inconclusive. The delayed onset of skin metastases led to the diagnosis of angiosarcoma, however the patient died from pleuropulmonary progression before treatment could be started. We review the literature of primary pleuropulmonary angiosarcoma and discuss its treatment modalities.

Primary pleural angiosarcoma associated with pneumoconiosis: An autopsy case

“…Therefore, a diagnosis of primary pleural angiosarcoma requires exclusion of other primary lesions, as was done in the present case. Although the lung is a common site of metastasis in the natural progression of angiosarcoma, the patient in our case presented with masses that were mainly located in the lower right pleura, and invasion into the lung parenchyma was not detected. Primary angiosarcoma can present as either a single mass or multiple nodules, along with diffuse thickening of the pleura .…”

“…Although the lung is a common site of metastasis in the natural progression of angiosarcoma, the patient in our case presented with masses that were mainly located in the lower right pleura, and invasion into the lung parenchyma was not detected. Primary angiosarcoma can present as either a single mass or multiple nodules, along with diffuse thickening of the pleura . In the current case, autopsy was performed only in the chest, restricting the search for other possible origins of the angiosarcoma; however, we note that abdominal CT did not show any signs of additional tumors, and we found no signs of malignancy on the skin.…”

“…Thus, for tumors that are focally positive for CKs (CK7, 14, 8, and 18), strongly positive for vimentin, and negative for mesothelial markers such as calretinin and CK5, a vascular tumor should be suspected. Subsequently, angiosarcoma can be confirmed with antibodies against endothelial markers such as CD31, CD34, and factor VIII‐related antigen, since this tumor shows positivity for at least one of these three markers.…”

“…Despite its rarity, it can involve the pleura and has an extremely aggressive clinical course. Pleural angiosarcoma mainly occurs in men and often presents with hemothorax, causing dyspnea and chest pain . Although the pathogenesis and etiology of pleural angiosarcoma are unclear, several risk factors, including cigarette smoking, a history of tuberculosis, and chronic pyothorax, have been hypothesized.…”

“…Pleural angiosarcoma mainly occurs in men and often presents with hemothorax, causing dyspnea and chest pain. [2][3][4][5] Although the pathogenesis and etiology of pleural angiosarcoma are unclear, several risk factors, including cigarette smoking, a history of tuberculosis, and chronic pyothorax, have been hypoth-esized. Chronic inflammation may also play a role in the development of angiosarcoma.…”