Normal plasma cells are found in the bone marrow and function as part of the immune system. In myeloma, plasma cells become cancerous causing tumor formations called plasmacytomas. Although rare, patients with myeloma can also have central nervous system (CNS) and pericardial symptoms. However, CNS and pericardial involvement has never been reported to occur simultaneously in patients with relapsed myeloma, until now. 

A recent case study published in Case Study Reports in Oncology follows the first known case of simultaneous central nervous system and pericardial involvement in a patient with high-risk multiple myeloma previously treated with chemotherapy and allogenic hematopoietic stem cell transplantation. “This case demonstrates a rare presentation of very rapidly relapsing multiple myeloma in the form of extraosseous, solitary plasmacytoma and malignant pericardial effusion, which has not been previously reported in the literature,” the authors report.

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The case follows a 54-year-old female with a diagnosis of IgG kappa multiple myeloma. Based on the International Staging System, she was classified with stage 1 disease. She was treated with several cycles of chemotherapy drugs and radiation therapy. After treatment, her IgG level was normal and her serum protein electrophoresis (SPEP), which previously showed an M-spike with monoclonal IgG kappa, was now too low to measure. A bone marrow biopsy revealed less than 1% clonal plasma cells. The patient underwent a reduced-intensity allogenic hematopoietic stem cell transplantation (HSCT) approximately 1 year after diagnosis.

However, 2 months after transplantation she developed plasmacytoma of the thoracic spine. Her bone marrow samples at that time showed complete donor engraftment and a lack of abnormal cells. Her SPEP showed low levels of monoclonal IgG kappa. She underwent radiation.

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Yet, 3 months after HSCT, she reported intractable nausea, vomiting, and a 30-lb weight loss. New thoracic soft tissue lesions were detected by spinal MRI, and a brain MRI revealed a mass in the extra-axial right occipital lobe that extended to the cerebellum. Pathology of the tumor revealed CD138-positive cells consistent with plasmablastic plasmacytoma.

During the same visit, a CT of the thorax revealed a large pericardial effusion. The patient did not report any cardiac or respiratory complaints and the effusion was monitored. However, it eventually enlarged in size, prompting immediate pericardial drainage in which CD138-positive plasma cells were also detected. Malignant pericardial effusion is rare and occurs in less than 1% of all myeloma cases.