Maria Coakley, Beeletsega Yeneneh, Allison Rosenthal, Rafael Fonseca, Farouk Mookadam

Multiple myeloma (MM) is characterized by a clonal proliferation of plasma cells. Although the bone marrow is the usual site of involvement, extramedullary plasmacytomas (EMPs) also occur, affecting any tissue. Cardiac and pericardial involvement, although described, have been rare occurrences. We present the case of a 61-year-old female patient 47 days after autologous stem cell transplant for MM who developed cardiac tamponade owing to extramedullary recurrence of myeloma, pulmonary embolism, and takotsubo cardiomyopathy. We performed a review of the published studies of all cases of MM presenting at diagnosis or relapse with cardiac or pericardial involvement in the past 25 years. Including our patient, 34 patients with plasmacytoma involving cardiac or pericardial structures were identified from the literature search. Approximately equal numbers of patients were male and female (42% and 57%, respectively). The mean age was 62 years. Primary plasmacytomas accounted for 12% of the cases. A history of MM, EMP, or monoclonal gammopathy of uncertain significance was noted in two thirds of the cases (66.6%). Treatment included chemotherapy and/or high-dose corticosteroids in 81.1% of cases and 27% underwent radiation therapy. The reporting of all cases to date has focused on unusual findings, rather than treatment approaches or new therapeutic strategies that might benefit patients. We suggest the formation of a database of all cases of cardiac and pericardial EMPs, with a focus on predictive disease variables, standardized staging, outcomes, and survival, to ensure that patients are optimally treated in the modern era.