Plasmablastic lymphoma (PBL) is an aggressive form of non-Hodgkin lymphoma associated with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV) infections.1,2 Prognosis is poor, with most patients succumbing to fulminant metastatic disease within the first year of diagnosis. The oral cavity is the most commonly reported location of PBL followed by the gastrointestinal tract, lymph nodes, and skin.3 We present a case of PBL presenting as a solitary nasal septal mass.
Report of a Case
An HIV-negative man presented with a 12-month history of left-sided nasal congestion and obstruction despite medical treatment. Physical examination revealed a large polypoid mass obstructing the left nasal cavity and computed tomographic imaging (Figure 1A and B) demonstrated a soft tissue polypoid density not involving the orbit or skull base. Preoperative magnetic resonance imaging (MRI) was not obtained because this lesion was isolated to the nasal cavity and benign in appearance. He was taken to the operating room for resection where the specimen was removed en bloc from the nasal septum (Figure 2A and B).
Figure 1.
Computed Tomographic Images Demonstrating Soft Tissue Mass Obstructing the Left Nasal Cavity
Figure 2.
Intraoperative Endoscopic Images of Patient’s Left Nasal Cavity With Polypoid Mass Obstructing View of the Middle Turbinate
The final pathologic analysis revealed EBV-positive PBL with CD138-positive, CD56-positive, CD20-negative, and PAX5-negative immunoprofile results. Although fluorescence in situ hybridization testing did not detect MYC translocation, gains of florescence signal were detected at multiple loci, including 3q27 (BCL6), 8q24 (MYC), and 14q32 (IGH), suggesting a complex karyotype. Further workup including MRI, positron emission tomographic (PET) scan, and bone marrow biopsy did not reveal any local or distant metastatic disease with final staging of stage 1 PBL with high-risk features. The patient elected to undergo 6 cycles of chemotherapy to include infusion with etoposide, vincristine, doxorubicin with bolus of cyclophosphamide, and prednisone with the addition of rituximab. After 1 round of therapy the patient developed neutropenic fevers so he was switched to cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab, which was well tolerated allowing for completion of chemotherapy. Posttreatment PET scan did not reveal any recurrent or active lymphoma, lymphadenopathy, or splenomegaly.
Discussion
Plasmablastic lymphoma is a rare aggressive large B-cell neoplasm, often associated with EBV infection.1 Originally described in 1997 in acquired immunodeficiency syndrome patients,2 PBL has also been described in immunosuppressed, posttransplant, and immunocompetent patients.4 A recent review3 identified 590 patients with PBL with a wide age range of effected individuals, with most individuals being adult males. Though the pathogenesis of PBL is incompletely understood, the cell of origin is theorized to be blastic proliferating B cells that have activated plasma cell gene expression programs.1 Both EBV infection and MYC oncogene dysregulation have been thought to contribute to the development of PBL where EBV infection has been shown to inhibit B-cell apoptosis and MYCtranslocation has been shown to impair the response to DNA damage through the loss of p53.3MYCrearrangement, as seen in approximately 50% of cases, is associated with decreased overall survival in HIV-positive patients with PBL.4
Survival for patients with PBL is low with the median survival being 3 and 4 months for HIV-positive and HIV-negative patients, respectively.5 Treatment is typically chemotherapy using cyclophosphamide, doxorubicin, vincristine, and prednisone with some studies reporting the use of etoposide. As was the case for this patient, recent studies have demonstrated possible improved complete response rates in patients receiving rituximab, although the mechanism remains unclear.6
Though PBL presents in the head and neck, most cases are identified in the oral cavity and gastrointestinal tract.2-4 Although reports exist of sinonasal PBL, to our knowledge this represents the first report of PBL originating from the nasal septum.
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Article Information
Published Online: July 18, 2019. doi:10.1001/jamaoto.2019.1719
Conflict Of Interest Disclosures: None reported.
Disclaimer: The views expressed in this article are those of the authors and do not reflect the official policy or position of the United States Army, Department of Defense, or the US Government.
Additional Contributions: We thank Shalini Dogra, MD, Kaiser Permanente–Mid Atlantic Permanente Group, for her assistance in providing care in this patient’s case.
References
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