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A woman in her 50s was admitted to the hospital with new left-sided nasal congestion, facial pain, and cheek numbness. Her medical history included kappa light chain multiple myeloma refractory to multiple chemotherapeutic regimens. She had been admitted 4 months earlier with similar symptoms of facial pain and sinus pressure. At that time, she was found to have persistent sinus mucormycosis, which required multiple rounds of surgical debridement. On this admission, her vital signs were stable, and she was afebrile. Laboratory test results showed a white blood cell count of 0.97 × 109 per liter with absolute neutrophil count of 650 × 106 per liter and platelet level of 17 × 109 per liter. A computed tomography scan showed complete opacification of the left paranasal sinuses (Figure, A). The patient underwent surgical debridement, and a prominent erythematous soft-tissue mass was seen in her left nasal vestibule (Figure, B). The nasal mass was resected for pathologic analysis (Figure, C).
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A. Extramedullary plasmacytoma
The patient’s presenting symptoms were similar to the previous presentation of mucormycosis and, given her immunocompromised state, were initially thought to be caused by recurrent invasive fungal sinusitis. However, the computed tomography scan showed evidence of a left maxillary soft-tissue abnormality concerning for plasmacytoma. In addition, the erythematous mass seen in the nasal vestibule had an appearance inconsistent with fungal sinusitis. Surgical pathologic results of the resected mass showed aggregates of plasmablastic forms with immunohistochemical staining for the plasma cell marker CD138, confirming the diagnosis of plasmacytoma.
Plasmacytomas are tumors composed of monoclonal immunoglobulin–producing plasma cells and most commonly present in bone (medullary plasmacytoma). However, they can also occur in soft tissue, as in this case (extramedullary plasmacytoma [EMP]).1,2 Medullary plasmacytomas occur in the axial skeleton (calvarium, spine, and rib cage) in 83% of cases.1 Most EMPs arise in the upper respiratory tract; specifically, 80% of solitary EMPs arise from the head and neck region.3
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Corresponding Author: Joel A. Sercarz, MD, Department of Head and Neck Surgery, David Geffen School of Medicine at University of California Los Angeles, 200 UCLA Medical Plaza, Ste 550, Los Angeles, CA 90095 (firstname.lastname@example.org).
Published Online: June 17, 2021. doi:10.1001/jamaoto.2021.1142
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient’s family for granting permission to publish this information.
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