Cyclophosphamide, Bortezomib and Dexamethasone (CyBordD) for Multiple Extramedullary Plasmacytomas and Bilateral Myelomatous Pleural Effusion

Multiple soft-tissue Extramedullary Plasmacytomas (EMP) and Myelomatous Pleural Effusion (MPE) are two uncommon manifestations of advanced Multiple Myeloma (MM). The presence of both multiple EMP and MPE in the same MM presentation is very rare. We report the case of an 81-year-old woman who presented with dyspnea on exertion and productive cough. Physical exam found bibasilar rales and multiple soft, subcutaneous, mobile lumps on her bilateral upper extremities. Computed Tomography (CT) of her chest with Intravenous (IV) contrast identified a large mantle of soft tissue surrounding the distal thoracic aorta, a second mass involving the anterior right chest wall, and bilateral pleural effusions. Subsequent biopsy of the right chest wall mass revealed plasma cell neoplasm. Pleural fluid cytology was consistent with plasmacytoid/plasmacytic neoplasm. She was started on Cyclophosphamide, Bortezomib and Dexamethasone (CyBorD). Repeat Positron Emission Tomography (PET)-CT after 11 days of treatment demonstrated excellent response with apparent resolution of the posterior mediastinal mass and right anterior chest wall mass, no activity in thoracic spine, and decreased bone activity. Despite improved disease activity, she acutely decompensated due to aspiration pneumonia and septic shock, and she expired one week later. EMP and MPE individually confer poor prognosis, and the presence of both conditions as the initial manifestations of MM may complicate the diagnostic process and suggest more aggressive clinical features. The experience of our patient suggests the potential for favorable outcomes to CyBorD if complications are avoided.