Case Presentation: Our patient was a 70-year-old male with a past medical history of HTN, HLD, COPD, and CAD status-post CABG. He originally presented with hypotension, new onset weakness, dyspnea, orthopnea and bilateral lower extremity pitting edema. As part of his work-up, a CT scan of the abdomen and pelvis was ordered, which revealed an 8-cm mass involving the majority of the right atrium, a 2.2 cm necrotic right pericardiophrenic lymph node and a 2.3 cm enhancing nodule from the right kidney. Additional labs revealed elevated liver function tests. Further workup involved a TEE which noted a right atrial mass. This mass was not present on a TEE done just a few months prior. A repeat CT confirmed a 10.8 cm right atrial mass that extended through the anterior atrial wall into the anterior mediastinum and pericardium as well as a small right-sided pleural effusion. Biopsies were obtained of both the atrial and renal masses. The renal mass was determined to be a renal oncocytoma that is to be treated in an outpatient clinic via cryoablation. Pathology of the atrial mass proved to be classic Hodgkin lymphoma, nodular sclerosis subtype, CD15 and CD30 positive. Bone marrow biopsy showed normal marrow with multilineage hematopoiesis and no cytogenic evidence of abnormal clone of cells, which was consistent with analysis of the pleural effusion. This enabled us to determine that the atrial mass was not a result of metastasis but rather an original presentation of classic Hodgkin lymphoma. The physical exam findings of bilateral lower extremity edema and low-normal baseline blood pressure, as well as lab evidence of elevated liver enzymes were the result of mass involvement of the right atrium with possibly impaired venous return and thus low cardiac output. The lymphoma was treated with adriamycin, bleomycin, vinblastine, dacarbazine chemotherapy.
Discussion: Primary cardiac neoplasms are relatively rare with a prevalence of 0.001-0.03%. Metastatic involvement of the heart is more than 20 times as common as primary lesions. Myxomas are the most common primary cardiac tumor, accounting for nearly 50% of cardiac neoplasms. Furthermore, myxomas usually involve the left side of the heart as right-sided myxomas account for (only) 8.6% to 20% of all cardiac myxomas.Primary cardiac lymphomas are rare neoplasms involving the heart and pericardium, predominantly in immunocompromised patients, with Non-Hodgkin lymphomas as the predominant type. Diagnosis of cardiac lymphomas is challenging as many of the presenting symptoms often mimic other cardiac conditions. In our study, we report a rare case of nodular sclerosing Hodgkin’s lymphoma in an elderly male presenting with a right atrial mass with mediastinal extension. Although few postmortem studies reported the rare involvement of the heart by Hodgkin’s lymphoma, to our knowledge, this is the first reported case of Hodgkin lymphoma with antemortem presentation of an intracardiac mass.
Conclusions: Primary cardiac tumors are rare, with the incidence ranging from 0.0017% to 0.33%. Most primary cardiac tumors are benign, making up 58–77% of all primary cardiac tumors. Malignant tumors of the heart are less common and comprise 24–42% of all primary cardiac tumors. The incidence of cardiac involvement in metastatic lymphoma ranges from 7.5 to 9.3% in one case series. In contrast, the incidence of primary cardiac lymphoma is exceedingly rare, ranging from 0.04% to 1.8% of all primary cardiac tumors. With such a rare condition and vague presentation, a broad and thorough workup is imperative.