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Nasal Mass in a Middle-aged Woman With Multiple Myeloma and Recurrent Fungal Sinusitis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

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 (jsercarz@mednet.ucla.edu).

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.

References
1.
Dores  GM , Landgren  O , McGlynn  KA , Curtis  RE , Linet  MS , Devesa  SS .  Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: incidence and survival in the United States, 1992-2004.   Br J Haematol. 2009;144(1):86-94. doi:10.1111/j.1365-2141.2008.07421.xPubMedGoogle ScholarCrossref
2.
Pham  A , Mahindra  A .  Solitary plasmacytoma: a review of diagnosis and management.   Curr Hematol Malig Rep. 2019;14(2):63-69. doi:10.1007/s11899-019-00499-8PubMedGoogle ScholarCrossref
3.
Agarwal  A .  Neuroimaging of plasmacytoma: a pictorial review.   Neuroradiol J. 2014;27(4):431-437. doi:10.15274/NRJ-2014-10078PubMedGoogle ScholarCrossref
4.
Bladé  J , Fernández de Larrea  C , Rosiñol  L , Cibeira  MT , Jiménez  R , Powles  R .  Soft-tissue plasmacytomas in multiple myeloma: incidence, mechanisms of extramedullary spread, and treatment approach.   J Clin Oncol. 2011;29(28):3805-3812. doi:10.1200/JCO.2011.34.9290PubMedGoogle ScholarCrossref
5.
Di Stadio  A , Gambacorta  V , de Crescenzo  S ,  et al.  Extramedullary nasal plasmacytoma arising after polyp excision and the role of the inflammation in tumor development: a case report.   Mol Clin Oncol. 2020;12(5):451-455. doi:10.3892/mco.2020.2007PubMedGoogle Scholar
6.
Wax  MK , Yun  KJ , Omar  RA .  Extramedullary plasmacytomas of the head and neck.   Otolaryngol Head Neck Surg. 1993;109(5):877-885. doi:10.1177/019459989310900517PubMedGoogle ScholarCrossref
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