Abstract
Cardiac tumors are extremely rare. Here, we report an unusual case of cardiac plasmacytoma that occurred 11 years after complete remission of the original multiple myeloma (MM). The tumor primarily manifested as a solitary extramedullary plasmacytoma (SEP) with extensive infiltration into the heart and large vessels. There was no evidence of systemic involvement. The relapsing tumor assumed a unique immunophenotype from CD138+/CD38+/CD56- to CD138-/CD38+/CD56-. The patient responded to chemotherapy consisting of carfilzomib, cyclophosphamide, and dexamethasone. This case highlights the importance of multimodal imaging evaluation and tissue diagnosis for accurately characterizing this rare disorder.
Highlights
We describe a case of relapsing cardiac plasmacytoma after chemotherapy and autologous stem cell transplantation presented as a solitary extramedullary plasmacytoma (SEP) invading the major intrathoracic cardiovascular structures
The incidence of primary cardiac tumors was reported to be less than 0.1% in a series of 12,000 autopsies
Diagnosis of SEP requires excluding the features of MM, including (1) free of anemia, hypercalcemia, or renal impairment; (2) PET/Computed tomography (CT) scan demonstrating no lytic bone lesions, and (3) absence of clonal plasma cells from bone marrow biopsy
Summary
The complete bone survey was negative for lytic bone lesions while bone marrow biopsy and flow cytometry revealed no monoclonal plasma cells. Despite negative bone marrow biopsy, the patient was diagnosed with MM (biopsy confirmed extramedullary plasmacytoma and bony lesions). She was treated with Revlimid and zoledronic acid for a short period of time. The patient responded favorably, as demonstrated by the shrinking mass and normalized serum IgG four months post-discharge. She remained largely asymptomatic after seven months
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