Here we describe a highly atypical case of primary plasmacytoma with extensive lymph node involvement. The vast majority of lymph node plasmacytomas are solitary lesions, with only seven documented cases involving multiple nodes. Primary lymph node plasmacytomas are exceedingly rare with fewer than 40 cases having been described in the literature to date. Primary plasmacytomas not involving bone (extramedullary plasmacytomas) typically manifest as lesions of the upper aerodigestive tract. The most common manifestation of primary plasmacytoma is as a single lesion of bone (solitary osseous plasmacytoma). Plasmacytomas are clonal proliferations of plasma cells that are cytologically and immunophenotypically indistinguishable from MM however, they demonstrate strictly local growth, without bone marrow or broader systemic involvement. The most common plasma cell malignancy is multiple myeloma (MM), which is characterized by a significant burden (> 10%) of clonal plasma cells within the bone marrow, monoclonal protein in serum and/or urine, and systemic symptoms (most commonly hypercalcemia, renal insufficiency, anemia, and/or bone lesions). Plasma cell neoplasms are characterized by the uncontrolled proliferation of malignant plasma cell clones. Importantly, we demonstrate that this entity may be treated with, and demonstrate excellent response to, systemic therapies often employed in multiple myeloma. This case report and accompanying literature review highlight the exceedingly rare and easily misclassified entity of primary plasmacytoma of diffuse lymph nodes. He remains in remission over 18 months following completion of therapy. He was treated with a myeloma-like regimen consisting of four cycles of bortezomib/dexamethasone followed by two cycles of thalidomide/prednisone with improvement in symptoms and near complete resolution of prior hypermetabolic lymphadenopathy. A bone marrow biopsy was unremarkable, and no monoclonal protein was identified, establishing a diagnosis of primary extramedullary plasmacytomas of the lymph nodes. A diagnosis of lymphoma was initially suspected however, a lymph node biopsy was consistent with plasmacytoma. Case presentationĪ 71-year-old Asian man presented with progressive fatigue in the setting of diffuse hypermetabolic lymphadenopathy throughout his chest, abdomen, and pelvis. Here we describe the most diffuse and widely distributed instance of primary lymph node plasmacytoma yet reported and present a description of its successful treatment with systemic therapy. Multifocal presentation in lymph nodes is nearly unheard of with only seven such cases reported in the existing literature, only four of which were diffuse enough to require systemic therapy. Because of their rarity, standardized treatment guidelines are not established, and treatment paradigms borrow heavily from those employed in multiple myeloma. Multifocal presentations are rare and are often not amenable to local treatment modalities, thus requiring systemic therapies. Because they are local phenomena, primary plasmacytomas are managed with local therapies such as radiation or, less commonly, excision. Presentation in a lymph node is very uncommon and can often be initially mistaken for lymphoma. Primary plasmacytomas most commonly manifest as solitary lesions of the bone or of the upper aerodigestive tract. Primary plasmacytomas are localized proliferations of clonal plasma cells occurring in the absence of a systemic plasma cell dyscrasia such as multiple myeloma.
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