Plasmacytoma (Solitary bone plasmacytoma, extramedullary plasmacytoma)

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Title: Plasmacytoma (Solitary bone plasmacytoma, extramedullary plasmacytoma)
Author: Rowell, Sean; Ho Zhi Guang, Matthew; Bianchi, Giada; Anderson, Kenneth C
Abstract: Plasmacytoma is a rare hematological malignancy characterized by a discrete, solitary mass of neoplastic monoclonal plasma cells that occurs either (1) inside (solitary bone plasmacytoma - SBP) or outside (extramedullary plasmacytoma - EMP) the bone. Plasmacytoma mainly affects people in the 5th to 6th decade of their lives, and the incidence of the disease is higher in men than in women. It also affects African Americans more often than Caucasians, with the lowest incidence being reported in Asian populations. Unlike MM, plasmacytoma is not a systemic disease and lacks the characteristic CRAB abnormalities such as hypercalcemia, renal failure, anemia, and bone disease, excluding the plasmacytoma itself. The median time to progression of plasmacytoma to MM is 2-3 years, with progression more likely to occur in SBP than EMP. SBP commonly presents in the axial skeleton and mostly manifests as local pain, pathological fractures nerve compression, while EMP is usually seen in the head and neck and typically manifests as space-occupying lesions. Therapeutically, local therapy such as radiotherapy (RT) is the gold standard and is able to achieve long-term disease-free survival in approximately 30% and 65% of patients with SBP and EMP respectively. Surgery (and adjuvant RT), on the other hand, is mostly reserved for easily resectable plasmacytomas.
Subject: Plasmacytoma; solitary bone plasmacytoma; extramedullary plasmacytoma; Leukaemia Section
Publisher: ARMGHM - Atlas Génétique des Cancers
Date: 2019

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