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.
Highlights
Angiosarcoma is a very uncommon neoplasm derived from endothelial cells
Primary pleural angiosarcoma is extremely rare; only 50 cases have been reported in the literature until 2010 [1,2,3]
We report a case of primary epitheloid angiosarcoma of the right pleura in a 70 years old man who had a two-weeks-history of progressive dyspnea and increase of abdominal lower limb volume
Summary
Angiosarcoma is a very uncommon neoplasm derived from endothelial cells. It represents 1% to 2% of all soft tissue malignancies. Imunohistochemical stains have been performed on serial paraffin sections of 5u mounted on precoated slides and followed by deparrafinization using the following antibodies: TTF1, calretinin, CKAE1/AE3, Ck5/6, CD3, CD20, CD31, CD 34, FVIII-related antigens, EMA, MPO, MELAN A, HMB45, HMBE1. It was a neoplastic proliferation consisting of large atypical epitheloid cells with abundant amphophilic cytoplasm. The tumor cells showed focal staining for EMA and for CKAE1/AE3 They were negative for HMBE1, CK5/6, HMB45, MELAN A, calretinin, CD3, CD20, MPO, and AML (Figure 4, Figure 5). The overall pathological immunohistochemical features supported the diagnosis of a malignant epitheloid vascular tumor consistent with epitheloid
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
