Key result
Primary right pleural epithelioid angiosarcoma precedes fatal pulseless VT arrest in a 70-year-old male.
Why the study?
Primary angiosarcoma of the pleura is an extremely rare tumor with an aggressive course and poor prognosis, and early diagnosis is challenging due to diverse clinical and radiological manifestations.
Case Report (n=1)
Highlights the diagnostic challenge of primary pleural epitheloid angiosarcoma, emphasizing the need for meticulous histological and immunohistochemical examination.
Alerts to possible sudden arrhythmic death after pleural angiosarcoma diagnosis; leaves open need for targeted surveillance in rare sarcomas.
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.
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Azzakhmam et al. (2019) conducted a case report in Primary epitheloid angiosarcoma of the pleura (n=1). Diagnosis of primary right pleural epitheloid angiosarcoma in a 70-year-old male was followed by death from pulseless ventricular tachycardia arrest one week after discharge.
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