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Review
. 2019 Aug 28:33:327.
doi: 10.11604/pamj.2019.33.327.18145. eCollection 2019.

Primary epitheloid angiosarcoma of the pleura: an exceptional tumor location

Mustapha Azzakhmam  1 , Abderrahim Elktaibi  1 , Mohamed Reda El Ochi  1 , Mohamed Allaoui  1 , Abderrahmane Albouzidi  1 , Mohamed Oukabli  1
Affiliations
Review

Primary epitheloid angiosarcoma of the pleura: an exceptional tumor location

Mustapha Azzakhmam et al. Pan Afr Med J. .

Abstract

Primary angiosarcoma of the pleura is an extremely rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by an aggressive course and a poor prognosis. The early diagnosis is challenging due to diverse clinical and radiological manifestations. We report a case of a 70 year old male with primary right pleural epitheloid angiosarcoma. The patient had a history of a two week's progressive dyspnea. CT-scan showed a prominent thikening of the right pleura associated with pleural effusion and atelectasis. CT-scan guided by biopsy was performed and histological examination showed a tumor proliferation consisting of sheets of polygonal and epitheloid cells showing rudimentary vascular differentiation. Immunohistochemically, tumor cells were strongly positive for CD31 and Factor VIII-related antigen, negative for CD34, weakly and focally positive for EMA and Cytokeratine. The overall pathological and immunohistochemical features of the pleural specimens supported the diagnosis of epitheloid angiosarcma. The patient died after a week of discharge by pulsless ventricular tachycardia arrest. In addition, we also present a brief litterature review on pleural angiosarcoma. Our experience with this case suggests that comprehensive and sufficient sample collection and meticulous histological examination aided with immunohistochemical stains, particulary the endothelial markers, are required for accurate diagnosis of this rare malignancy.

Keywords: Angiosarcoma; Pleura; epitheloid; immunohistochemistry.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Chest radiography (X-ray) showing abundant right thorax effusion
Figure 2
Figure 2
CT-scan showing a prominent thickening of the right pleura
Figure 3
Figure 3
(HEX20); malignant proliferation of large epitheloid cells with marked nuclear pleomorphism and prominent nucleoli
Figure 4
Figure 4
(a) diffuse strong staining with CD31; (b) diffuse strong staining with Factor VIII related antigens; (c) the negative staining of the tumoral cells with CD34; (d) weakly focal staining with EMA (magnification power x20)
Figure 5
Figure 5
Focal cytoplasmique staining of tumoral cells with cytokeratin AE1/AE3, [HEx40]
See this image and copyright information in PMC

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