Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma

Most patients with plasma cell neoplasia have generalized disease at diagnosis, i.e. multiple myeloma (MM). However, a minority (<5%) of patients with plasma cell malignancies present with either a single bone lesion, or less commonly, a soft tissue mass, of monoclonal plasma cells: solitary bone plasmacytoma (SBP) or extramedullary plasmacytoma (SEP). SBP has a high risk of progression to MM and on magnetic resonance imaging (MRI) examination at least 25% of patients with an apparent solitary lesion have evidence of disease elsewhere (Moulopoulos et al, 1993). In contrast, SEP is nearly always truly localized and has a high cure rate with local treatment. The diagnosis and management of patients with solitary plasmacytoma requires the same range of clinical and laboratory expertise as for patients with MM (UK Myeloma Forum, 2001). The primary treatment for most patients will be radiotherapy, but surgery may also be required, where close liaison among the haematologist, radiotherapist and surgeon is crucial for planning optimum care. Methods A literature search was performed by a professional librarian using MEDLINE and EMBASE from 1996 to March 2002. A search was made for randomized-controlled trials involving plasmacytoma, papers where plasmacytoma was the major focus of the paper and reviews where plasmacytoma was the major focus. The literature was then reviewed by the subgroup of the Guidelines Working Group of the UK Myeloma Forum. Levels of evidence and grades of recommendation are shown in Table I. SBP and SEP are rare diseases and most of the evidence relates to retrospective data from patient series collected over long periods of time. Very few formal clinical trials have been performed. The majority of the recommendations given are therefore based on consensus of expert