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Tony Mathews, Rachel Bring, Azkia Khan, Itzhak Kronzon, Eugenia Gianos, A stepwise diagnostic approach to superior vena cava syndrome, European Heart Journal - Cardiovascular Imaging, Volume 20, Issue 3, March 2019, Page 367, https://doi.org/10.1093/ehjci/jey185
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A 44-year-old man was referred to our hospital with several months history of engorged veins in the right upper chest wall and no complaints. The plain chest X-ray (CXR) (Panels A and B) revealed normal lung fields, prominent azygos vein, and obliteration of the retrosternal space on lateral view. Transthoracic echocardiogram (TTE) (Panel C) demonstrated a large (6 × 6 cm) homogeneous mass which nearly filled the right atrial (RA) cavity. Computed tomography (CT) (Panel D) showed that the mass extended to the superior vena cava (SVC), both subclavian veins and to the inferior vena cava. The azygos vein was markedly dilated with extensive venous collaterals noted between the superior and inferior vena cava. Magnetic resonance imaging (MRI) (Panels E, F, G) confirmed that the mass was consistent with tumour as it was hypointense on T1-weighted imaging, slightly hyperintense on T2-weighted imaging and showed patchy enhancement on delayed-contrast imaging. Biopsy of the mass via right heart catheterization (femoral approach) demonstrated atypical large cells with irregular nuclear contour and prominent nucleoli consistent with diffuse large B cell lymphoma of follicular origin based on tumour markers (Panels H and I). A trial of chemotherapy (rituximab, doxorubicin, vincristine, and prednisone) shrunk the tumour to half its size within 1 month and it was no longer visible on follow-up imaging after six cycles of chemotherapy (Panel J). The patient is now 6 years post-treatment and still in remission proving that an escalating work-up with cardiac imaging followed by biopsy can preclude unnecessary high-risk surgery.