Castleman disease infiltrating great vessels and right atrium.

Castleman disease, or angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder of unknown etiology and pathogenesis. Its importance lies in the fact that it has been associated with human immunodeficiency virus (HIV) and human herpes virus 8, as well as other neoplastic diseases such as Kaposi’s sarcoma, lymphomas, and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes). We present the case of a 66-year-old man with no known cardiovascular risk factors who complained of dyspnea upon moderate exertion, together with a weight loss of 4 to 5 kg over a 6-month period. In the physical examination, a grade 2/6 systolic murmur was detected at the pulmonary site (left second intercostal space) on auscultation. A chest X-ray showed no evidence of cardiomegaly or signs of pulmonary congestion. Transthoracic echocardiography revealed the presence of mild pulmonary stenosis with a peak pressure gradient of 23 mmHg and, on the parasternal short-axis view, there was a marked concentric thickening of the ascending aorta, which exerted pressure on the pulmonary artery. In view of the echocardiographic findings, we performed a magnetic resonance study (Figs. 1 and 2), which revealed a soft tissue mass surrounding the aortic root and ascending thoracic aorta and occupying the lumen