Objective:To describe an intranasal mass initially diagnosed and treated as benign that eventually turned out to be a malignant extramedullary plasmacytoma of the maxillary sinus and to review the literature on its presenting signs and symptoms, diagnosis, management and pathophysiology. Methods: Design: Case Report Setting: Tertiary Public Hospital Patient: One Results: A 45-year-old male with persistent nasal obstruction and intermittent epistaxis underwent several biopsies of a mass shown on computed tomography scans as heterogeneously enhancing, expansile, occupying the left maxillary sinus with extension into the left nasal cavity with areas of erosion. Immunohistochemical staining was negative for cytokeratin (CK) and leukocyte common antigen (LCA). Complete excision yielded a final histopathologic interpretation of plasmacytoma. Laboratory examinations excluded multiple myeloma. The final diagnosis was extramedullary plasmacytoma and he was treated with post-operative adjuvant radiotherapy. Conclusion: Plasmacytoma may present in the sinu-nasal region and be part of a systemic disease like multiple myeloma. A high index of suspicion and thorough initial histopathological work-up may help in establishing a definitive diagnosis and providing optimum treatment.

Anaplastic large cell lymphoma (ALCL) is a rare subtype of T-cell non-Hodgkin lymphoma (NHL) primarily involving the lymph nodes; however, extra-nodal manifestations are also common. Diagnosis can be confirmed by a combination of histopathology and immunohistochemical staining. Complete workup and staging include imaging and bone marrow examination. This presents a case of a 55-year-old male with anaplastic lymphoma kinase (ALK) - negative ALCL presenting with an alar mass. ALCL patients often present with rapidly progressing lymphadenopathy. Extra-nodal manifestations commonly involve the skin, liver, lung, and gastrointestinal tract. Biopsy of the mass showed small to medium-sized anaplastic lymphoid cells that stained positive for CD30, LCA (CD45), CD99, and negative for CD20, ALK (CD246), neuron-specific enolase, CD34, CD5, PAX5, TdT, MPO, CD138, EMA, pancytokeratin, CD3 and synaptophysin. These findings were most compatible with an ALK-negative ALCL. The patient was started on a combination of brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone (BV + CHP) every 21 days for 6 cycles. There was a progressive decrease in the size of the mass, and a resolution was noted after the 5th cycle. FDG-PET/CT scan was done after the 6th cycle of chemotherapy and 6 months after completion of treatment. Both scans showed no evidence of metabolically active nodal or extra-nodal lymphomatous disease. This case showed a unique extra-nodal manifestation of an ALK-negative ALCL presenting as an alar mass with a good response to BV + CHP. However, more evidence is necessary to further establish the role of BV as the first-line treatment regimen for CD30-positive peripheral T-cell lymphoma (PTCL), including ALK-negative ALCL.
lymphoma ; anaplastic large cell lymphoma ; brentuximab vedotin ; case report