Epithelioid Angiosarcoma of the Pleura: A Case Report

Abstract

Angiosarcomas of the pleura are very rare tumors and it is difficult to differentiate them from other common pleural tumors such as mesothelioma and metastasic carcinoma clinically and pathologically. We report a case of a young Korean woman with angiosarcoma arising in the pleura. A 34-yr-old woman presented with dyspnea and chest tightness and pain for several months. A computed tomographic scan of the chest showed diffuse thickening of the left pleura and effusion with passive atelectasis. At thoracotomy the left pleura was thick and indurated. Histologically, the decorticated pleura revealed infiltration of sheets or cords of polygonal and epithelioid tumor cells showing rudimentary vascular differentiation. Immunohistochemically, the tumor cells were strongly positive for CD31, CD34, and vimentin, whereas weakly positive for factor VIII, and negative for cytokeratin, which are characteristic and specific findings of angiosarcoma.

Primary Pleural Angiosarcoma in a 63-Year-Old Gentleman

“…Primary pleural angiosarcomas are exceptionally rare. As of 2010, only around 50 case reports of primary pleural angiosarcomas have been documented in the English literature so far [4]. Average age at presentation is 57 years, and males are affected more frequently than females (male-female ratio is 9 : 1) [6].…”

“…Primary pleural angiosarcomas are extremely rare. As of 2010, only around 50 case reports have been documented in the literature [4]. Herein, we report a case of primary epithelioid angiosarcoma of the right pleura in a 63-year-old gentleman who presented with a 3-month history of right-sided chest pain, dyspnea, and hemoptysis.…”

Problems in mesothelioma diagnosis

“…Some overlap exists, as the latter may also have epithelioid features, and in the pleura it may be difficult to make a clear distinction between the two. Both may present with pleural effusion and diffuse pleural thickening and are capable of simulating mesothelioma clinically and radiologically, providing a further trap for the unwary histopathologist 89–92 …”

Pancoast tobias syndrome revealing primary epithelioid pleuro-pericardial angiosarcoma: A case report and review of the literature

“…Different histological types of pleural tumors are found in the World Health Organization (WHO) classification 2004, including mesothelioma (the most frequent neoplasm), lymphoma and mesenchymal tumors such as epithelioid haemangioendothelioma, angiosarcoma, synovial sarcoma, solitary fibrous tumor, calcifying tumor and desmoplastic small round cell tumor 2 . Twenty nine cases of primary pleural AS have been reported in the literature [5][6][7][8][9][10] . A strong link between pleural AS and tuberculous-related chronic pyothorax has been reported in Japanese patients 9 .…”