Guidelines on the Diagnosis and Management of Solitary Plasmacytoma of Bone (SBP) and Solitary Extramedullary Plasmacytoma (SEP)

British Committee for Standards in Haematology

Table of Contents

Compiled by a subgroup of the Guidelines Working Group of the UK Myeloma Forum on behalf of the British Committee for Standards in Haematology (BCSH) - a sub-committee of the British Society for Haematology (BSH):

Published on UKMF website: Summer 2003
Most recent minor revision: [none]

Planned full revision date Summer 2006; interim updates will be published on the UKMF and BSH/BCSH web-sites, as they occur.

Working and compiling group:

• Dr Jenny Bird, Consultant Haematologist
• Dr Graham Jackson, Consultant Haematologist
• Dr Helen Lucraft, Consultant Clinical Oncologist
• Mr Anthony Reece, Consultant Orthopaedic Surgeon
• Dr Diana Samson, Chairman UK Myeloma Forum
• Dr Richard Soutar, Consultant in Haematology and Transfusion Medicine
• Mr Eric Low, International Myeloma Foundation UK (patient advocate group)

Written with the support of an educational grant from the International Myeloma Foundation UK

Address for Correspondence

Dr Richard Soutar, UKMF Administration Office, Department of Haematology, Guys' Hospital, London SE1 9RT

Disclaimer

The advice and information in these guidelines is believed to be true and accurate at the time of going to press. However the authors, the British Society of Haematology, the UK Myeloma Forum and the publishers accept no legal responsibility for the content of these guidelines. See also disclaimer on main Guidelines page.

Acknowledgement

We are grateful to Ms A Thain, Librarian, Beatson Oncology Centre, Glasgow for assistance with literature searches.

Table of Contents

1. Introduction
2. Methods
3. Solitary Bone Plasmacytoma (SBP)
   • Context
   • Diagnosis and investigation of SBP
   • Treatment of SBP
4. Solitary Extramedullary Plasmacytoma (SEP)
   • Context
   • Diagnosis and investigation of SEP
   • Treatment of SEP
5. Patient Information and Support
6. Tables
7. References

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1. Introduction

Most patients with plasma cell neoplasia have generalised disease at diagnosis, i.e. multiple myeloma (MM). However, a minority (less than 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 plasmacytoma of bone (SBP) or extramedullary plasmacytoma (SEP). SBP has a high risk of progression to MM and on 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 localised 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 multiple myeloma (UK Myeloma Forum, 2001). The primary treatment for most patients will be radiotherapy, but surgery may also be required, and close liaison between haematologist, radiotherapist and surgeon is crucial for planning optimum care.

2. Methods

A literature search was performed by a professional librarian using MEDLINE and EMBASE from 1996 to March 2002. A search was made for randomised 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 Guidelines subgroup listed above. 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 opinion.