TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Fellow Case Reports INTRODUCTION: Extramedullary plasmacytoma (EMP) are rare manifestations of multiple myeloma (MM). We report a case of pulmonary EMP presenting as hilar mass. CASE PRESENTATION: A 51 year old female presented with hearing loss and right facial palsy. Medical history was significant for MM diagnosed 2 years prior, treated with bortezomib-prednisone therapy. 6 months ago, thoracic spine plasmacytoma were found requiring surgical resection and local radiation. Remission was perceived and she was maintained on Thalidomide. MRI brain and spine revealed meningeal enhancement with lytic bony lesions, concerning for metastatic disease. CT chest abdomen pelvis revealed a 4.5 x 5.5 cm right hilar mass with complete right lower lobe (RLL) bronchus occlusion, post-obstructive pneumonia, right pleural effusion, pleural thickening and nodularity. She underwent bronchoscopy with endobronchial biopsy of RLL bronchial nodules and EBUS guided transbronchial needle aspiration of right hilar mass. Pathology was consistent with plasma cell myeloma with plasmablastic features. Given her MM history and absence of EBV, primary plasmablastic lymphoma was less likely. A bone marrow biopsy showed >10% myeloma cells, thus her diagnosis was consistent with relapsed MM with extramedullary plasmacytoma (EMP) rather than primary pulmonary plasmacytoma (PPP). Subsequently she underwent systemic and intrathecal second line chemotherapy, but her course was complicated by rapidly progressive refractory disease and patient chose to pursue hospice care. DISCUSSION: EMP are seen in 7 % patients with MM at the time of diagnosis, whereas another 6% develop it later in the course of illness; these portend poor prognosis. Among them, pulmonary EMP are extremely rare. Other pulmonary complications of multiple myeloma include pneumonia, including organizing pneumonia like consolidation, multiple pulmonary nodules, interstitial infiltrates and pleural thickening and effusion. In contrast, primary solitary extramedullary plasmacytoma is a rare discrete plasma cell neoplasm within soft tissue, without bone marrow involvement or other systemic features of MM. They commonly occur in the aero-digestive tract, of which solitary PPP is a rare type. PPP have better prognosis and longer survival rates compared to EMP associated with MM. Their management also differs, EMP associated with MM are treated aggressively with chemotherapy and local radiation or surgery as needed, whereas solitary PPP are treated with radiation only. CONCLUSIONS: Knowledge of pulmonary EMP and its clinical course is important for physicians, such that the etiology of pulmonary abnormalities in patients with multiple myeloma are evaluated and determined in order to ensure appropriate therapy and patient counseling. REFERENCE #1: Bladé J, et al. Soft-tissue plasmacytomas in multiple myeloma: incidence, mechanisms of extramedullary spread, and treatment approach. J Clin Oncol. 2011;29(28):3805-3812 REFERENCE #2: Varettoni M, Corso A, Pica G, Mangiacavalli S, Pascutto C, Lazzarino M. Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: a longitudinal study on 1003 consecutive patients. Ann Oncol. 2010;21(2):325-330. doi:10.1093/annonc/mdp329 REFERENCE #3: Prasad R, Verma SK, Sodhi R. Multiple myeloma with lung plasmacytoma. Lung India. 2011 Apr-Jun; 28(2): 136-138. DISCLOSURES: No relevant relationships by Jyotirmayee Lenka, source=Web Response No relevant relationships by Naman Sharma, source=Web Response
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