Rheumatism as a cause of cardiac hemangioma: a rare case report and review of literature with special focus on etiology

Abstract

Background: Cardiac hemangioma is a very rare benign tumor of the heart which accounts for 1-2% of all primary cardiac tumors. Multiple cardiac hemangiomas are even rarer with only three cases published in the literature. Pathologically it can be divided into cavernous hemangioma, capillary hemangioma, arteriovenous hemangioma, mixed-type hemangioma, and so on. At present, the etiology of cardiac hemangioma is not completely clear. In this study, we present multiple cardiac hemangiomas located in the right atrium and discuss the new unreported possible cause (rheumatism) of cardiac hemangioma. This is the fourth case of multiple cardiac hemangiomas in the medical literature and the first time to present rheumatism as the cause of cardiac hemangioma.

Case presentation: A 53-year-old man presented to the clinic with intermittent chest tightness and shortness of breath for 2 years. On echocardiography, multiple soft tissue masses in the right atrium were found. The patient had rheumatic heart disease with severe mitral stenosis and moderate tricuspid regurgitation. Two masses with a diameter of about 20 mm and 15 mm were seen in the right atrium. One mass was located on the inferior margin of the fossa ovalis and the other was adjacent to the inferior vena cava. Both masses were successfully removed surgically. The mitral valve replacement and tricuspid valve plasty were performed at the same time. The postoperative histopathology results confirmed the diagnosis of cavernous hemangioma.

Conclusion: The occurrence of multiple hemangiomas in the heart is possible, especially in the presence of rheumatism. Rheumatism is one of the possible etiologies of cardiac hemangioma. Cardiologists and cardiac surgeons should be aware of its occurrence and should consider cardiac hemangioma as a differential diagnosis especially in rheumatic heart disease patients when they present with soft tissue cardiac masses for accurate management.

Keywords: Benign heart tumors; Cardiac surgery; Hemangioma; Rheumatic heart disease; Rheumatism.

Fig. 1

Transthoracic echocardiography images of hemangioma, (A) Two hyperechoic masses in the right atrium (red arrow), (B) Preoperative echocardiography, (C) Postoperative echocardiography

Fig. 2

CT examination of hemangioma, (A) Abnormal structure in the right atrium by plain CT (red arrow), (B) Contrast-enhanced arterial phase, (C) Coronal view, (D) Contrast-enhanced venous phase, (E) Sagittal view

Fig. 3

(A) Intraoperative view of the hemangioma, (B) Specimen of the resected mitral valve leaflets (C) Specimen of the hemangioma

Fig. 4

Histopathology of cardiac hemangioma, (A) (HE×40): The structure of hemangioma is complete without multinucleated cells, (B) Calcification can be seen on HE stain at the base of hemangioma, (C) CD34 immunohistochemical staining: CD34+, (D) Masson staining: The capsule’s wall structure is complete, (E) Reticular fiber staining: The vessel wall structure is complete, (F) HE: Degenerative lesions at the base of hemangioma can be seen