Background:Primary cardiac tumors are infrequently, usually benign, and diagnosed incidentally. Among primary cardiac tumors, cardiac hemangiomas are exceptionally rare with only a 1–2% incidence. In most cases, histological diagnosis is made after surgical removal or at autopsy.
Case presentation: A 36-year-old male patient is suffering from several months of bothering night sweats. He has no previous medical history and is not taking medication. After performing various exams, including an electrocardiogram and chest rx-ray that were unremarkable, the patient performed a chest TC scan that shows a large mass at right atrial level (Figure 1). Surprisingly, at transthoracic echocardiography the mass was not visible and moreover the exam was normal. To better visualize the mass, a transesophageal echocardiography was performed, showing a inhomogeneous, round-shape, intracavitary mass of 70×65 mm in size. The mass appear to be fixed, expansive, with no embolic-features and seem to be initially-obstructing the upper-cava blood flow (Figure 2), even the patient was hemodynamically stable. A diagnosis of lymphoma was initially suspected and an Total-body PET-TC was performed, showing that the heart mass was metabolically inactive and an intestinal metabolic active mass. Intestine mass biopsy, made by colonscopy, showed a non-malignous villous adenoma. Before undergoing heart surgery, to understand the nature of the mass, we performed a intracavitary ecocardiography (ICE)-guided percutaneous biopsy. Histologically, the heart tumor was composed of cavernous channels embedded in a loose fibrous stroma, compatible with Cavernous hemangioma. At heart surgery, the mass was localized inside the right atrium, at upper-front level, adhered to a small part of atrial myocardium, and was removed without complications. The right atrium was rebuilt with artificial patch. (Figure 3).
Conclusions: Cavernous cardiac Hemangioma is a rare tumor that may have an atypical clinical presentation. To our knowledge, this is the first case of cardiac hemangioma diagnosed by ICE-guided endomyocardial biopsy.