European Heart Journal - Cardiovascular Imaging Advance Access published February 3, 2015

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doi:10.1093/ehjci/jeu324

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Unexplained bloody pericardial effusion: a diagnostic dilemma Adil Rajwani1,2*, Dusan Kotasek3,4, Robert Xu1,2, Karen Teo1,2,3, and Matthew Worthley1,2,3,5 1 Department of Cardiology, Royal Adelaide Hospital, North Terrace, Adelaide, South Australia 5000, Australia; 2Department of Cardiothoracic Surgery, Royal Adelaide Hospital, North Terrace, Adelaide, South Australia 5000, Australia; 3Discipline of Medicine, University of Adelaide, Adelaide, South Australia 5000, Australia; 4Adelaide Cancer Centre, Adelaide, South Australia 5037, Australia; and 5South Australian Health and Medical Research Institute, Adelaide, South Australia 5000, Australia

* Corresponding author. Tel: +61 8 82224000; Fax: +61 8 82222454, E-mail: [email protected]

Conflict of interest: None declared. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2015. For permissions please email: [email protected].

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A 38-year-old Caucasian male smoker presented with dyspnoea secondary to a large pericardial and moderate left pleural effusion, with elevated serum inflammatory markers and bloody pericardial aspirate. Large-volume fluid samples proved negative for malignancy and acid-fast bacilli, both on first presentation and at recurrence 1 month later. No malignancy was apparent on contrast computed tomography, and treatment with colchicine and prednisolone was initiated. No further re-accumulation was observed at 2 months; serum inflammatory markers had normalized and prednisolone was tapered accordingly. However, new unilateral retro-orbital pain 3 months after initial presentation heralded the finding of a large invasive tumour mass eroding the right petrous temporal bone, with histological identification of angiosarcoma at urgent resection. Re-assessment now by [18F]FDG/PET-CT exhibited intense tracer activity at the right cardiac atrium (Panel A and B). Further evaluation by cardiac MRI demonstrated a right atrial mass (Panel C; see Supplementary data online, Video S1) and extensive irregular lesions in the pericardial space with myocardial invasion (Panel D and E; see Supplementary data online, Videos S2 and S3). Gadolinium contrast uptake was surprisingly modest, limited primarily to the right atrial mass and the lateral aspect of the left ventricle (Panel F–H; see Supplementary data online, Video S4) with no late gadolinium enhancement. Nonetheless, surgical exploration confirmed extensive invasive and necrotic tumour not amenable to novel treatment with auto-transplantation (Panel I). Histological analysis confirmed Grade III angiosarcoma (Panel J), and the patient died shortly after. Given the significant likelihood of malignancy in the setting of unexplained bloody effusion with tamponade, early consideration of functional imaging is warranted even when anatomical imaging and cytology are ostensibly reassuring. Panel (A and B) Coronal and axial [18F]FDG/PET-CT reveal intense tracer activity at the right atrial wall. (C –E) Steady-state free precession cardiac MRI in modified right atrial, LVOT, and HLA views. A white arrow identifies the right atrial mass. (F –H ) Phase-sensitive inversion recovery MRI acquired for early gadolinium enhancement. (I ) Extensive tumour within the pericardial space is confirmed on surgical drainage and debulking. (J ) Histological analysis (haematoxylin –eosin, ×40) confirms angiosarcoma, with plump spindled and pleomorphic epithelioid cells forming solid sheets, ramifying anastomosing vascular channels and papillary formations.

Unexplained bloody pericardial effusion: a diagnostic dilemma.

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