Primary pleural angiosarcoma in a 63-year-old gentleman

Abstract

Primary pleural angiosarcomas are extremely rare. As of 2010, only around 50 case reports have been documented in the literature. Herein, we report the case of a 63-year-old gentleman who presented with a 3-month history of right-sided chest pain, dyspnea, and hemoptysis. Chest X-ray showed bilateral pleural effusion with partial bibasilar atelectasis. Ultrasound-guided thoracocentesis showed bloody and exudative pleural fluid. Cytologic examination was negative for malignant cells. An abdominal contrast-enhanced computed tomography (CT) scan showed two right diaphragmatic pleural masses. Whole-body positron emission tomography/computed tomography (PET/CT) scan showed two hypermetabolic fluorodeoxyglucose- (FDG-) avid lesions involving the right diaphragmatic pleura. CT-guided needle-core biopsy was performed and histopathological examination showed neoplastic cells growing mainly in sheets with focal areas suggestive of vascular spaces lined by cytologically malignant epithelioid cells. Immunohistochemical analysis showed strong positivity for vimentin, CD31, CD68, and Fli-1 markers. The overall pathological and immunohistochemical features supported the diagnosis of epithelioid angiosarcoma. The patient was scheduled for surgery in three weeks. Unfortunately, the patient died after one week after discharge secondary to pulseless ventricular tachycardia arrest followed by asystole. Moreover, we also present a brief literature review on pleural angiosarcoma.

Figure 1

A chest plain radiograph (X-ray) showing bilateral pleural effusion, partial bibasilar atelectasis, and linear pleural calcifications involving the right lung.

Figure 2

An axial chest, abdominal, and pelvic contrast-enhanced computed tomography (CT) scan showing bilateral pleural effusion, partial bibasilar atelectasis, and peripheral dense calcifications of the right lung. In addition, two pleural masses touching the right hemidiaphragm were identified and measured 3.2 × 2.1 cm and 4.3 × 4.8 cm (white arrows). The masses were suspicious for neoplastic lesions. No axillary or mediastinal lymphadenopathy was identified. Furthermore, no evidence of distant metastasis was identified.

Figure 3

An axial chest positron emission tomography/computed tomography (PET/CT) scan showing two hypermetabolic fluorodeoxyglucose- (FDG-) avid mass lesions involving the right diaphragmatic pleura, suggestive of neoplastic lesions (white arrows).

Figure 4

(a) Histopathological examination of the right diaphragmatic pleural masses (magnification power, 40x) showing the neoplastic cells that grew mainly in sheets with focal areas suggestive of vascular spaces lined by cytologically malignant epithelioid cells. The neoplastic proliferation consisted of highly atypical large epithelioid cells with abundant eosinophilic cytoplasm, round to oval nuclei with marked pleomorphism, vesicular chromatin, and prominent single nucleoli. (b) Focal hemorrhagic areas were identified.

Figure 5

Immunohistochemical examination of the resected right diaphragmatic pleural masses. (a) Tumor cells stained positive for vimentin (magnification power, 40x). (b) Tumor cells stained positive for CD31 (magnification power, 40x). (c) Tumor cells stained positive for CD68 (magnification power, 40x). (d) Tumor cells stained positive for Fli-1 (magnification power, 40x).