Image Description
The presenting symptoms of primary cardiac lymphomas usually mimic other non-neoplastic cardiac conditions and may include chest pain, dyspnea, heart failure, arrhythmias, and pericardial effusion. Right side of the heart is more frequently involved and the tumor is usually centered in the myocardium. Transesophageal echocardiography (TEE) is the imaging modality of choice. Diagnosis can be made by open biopsy, endomyocardial biopsy, or percutaneous biopsy with TEE imaging.
Most primary cardiac lymphomas are diffuse large B-cell lymphomas (DLBCL). The example shown here is composed predominantly of centroblasts. The immunophenotypic and genetic features are similar to DLBCLs occurring in other sites.
About the Disease
Primary cardiac tumors are extremely uncommon. Secondary tumors (metastases to heart) are 20-50 times more common than primary cardiac tumors and yet still relatively rare. Of primary cardiac tumors, almost 75% are benign and the remaining 25% are malignant.Benign Primary Cardiac Tumors: Myxoma (50-70% of cases), rhabdomyoma, lipoma and lipomatous hypertrophy of interatrial septum, papillary fibroelastoma, and fibroma. Angiomas and teratomas are found mainly in children. Malignant Primary Cardiac Tumors: Mostly sarcomas, including angiosarcoma (30%), rhabdomyosarcoma (20%), leiomyosarcoma, liposarcoma, osteosarcoma, fibrosarcoma, and undifferentiated pleomorphic sarcoma. Other tumors include mesothelioma and lymphoma.Secondary cardiac tumors are far more frequent than primary cardiac tumors. The most common primary sites include melanoma, lung, breast, kidney, and lymphomas. Pericardium is usually affected, resulting in pericardial effusion.Cardiac tumors may be asymptomatic and found incidentally or present with obstructive, embolic, arrhythmic or systemic symptoms, depending upon the size and location of the mass. References: 1. Miller, D. V. & Revelo M. P. (2023). Diagnostic Pathology - Cardiovascular. 3rd Edition, Elsevier.2. Sheppard, M. N. (2022). Practical Cardiovascular Pathology 3rd Edition, CRC Press.3. Maximilian B. & Butany J. (2022). Cardiovascular Pathology 5th Edition, Academic Press.