Primary epithelioid angiosarcoma of the pleura: a case report and review of literature

Abstract

Primary angiosarcoma of the pleura is an extremely rare malignancy. Herein, we report the case of an elderly Chinese patient with primary left pleural epithelioid angiosarcoma. The 76-year-old man presented with a 4-month history of a cough with sputum expectoration and weight loss of 4 kg within one month. A chest scan showed a massive oval-shaped mass in the left pleural cavity. We then performed a left thoracotomy for tumor resection and surgical exploration. Histological examination of the resected specimen showed few viable tumor cells with significant atypia; tumor cells had large nuclei and prominent nucleoli and were arranged in a crack-like, sheeted pattern. Moreover, there was a significant amount of fibrinous exudates, hemorrhage, degeneration, and necrosis. With immunohistochemical analysis, tumor cells had strong expression of CD31, CD34, FLI-1, vimentin. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma.

Keywords: Primary angiosarcoma; immunohistochemistry; pleura.

Figure 1

A chest computed tomography scan showed a massive oval mass in the left pleural cavity.

Figure 2

The angiosarcoma tissues were stained with hematoxylin and eosin. A: There were scanty viable tumor cells in the periphery of the tissue block (hematoxylin and eosin [H&E] stain, ×40 magnification). B, C: Tumor cells were arranged in a crack-like and sheeted pattern (H&E stain, ×200). D: Tumor cells had large nuclei with prominent nucleoli (H&E stain, ×400).

Figure 3

The angiosarcoma tissues were analyzed by immunohistochemistry. The tumor cells were strongly positive for CD31 (A), CD34 (B), FLI (C), vimentin (D ×200), and Ki-67 (F), and were weakly focally positive for cytokeratin (G).