Elsevier

Clinical Oncology

Volume 16, Issue 6, September 2004, Pages 405-413
Clinical Oncology

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

https://doi.org/10.1016/j.clon.2004.02.007Get rights and content

Introduction

Most patients with plasma cell neoplasia have generalised disease at diagnosis (i.e. multiple myeloma [MM]). However, a few patients (less than 5%) 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 magnetic resonance imaging (MRI) examination, at least 25% of patients with an apparent solitary lesion have evidence of disease elsewhere [1]. 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 [2]. 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.

Section snippets

Methods

A literature search was carried out by a professional librarian using MEDLINE and EMBASE from 1996 to March 2002. A search was made for randomized controlled trials involving plasmacytoma, papers in which plasmacytoma was the major focus, and reviews in which plasmacytoma was the major focus. The literature was then reviewed by the Guidelines subgroup. Levels of evidence and grades of recommendation are shown in Table 1. SBP and SEP are rare diseases, and most of the evidence relates to

Epidemiology and clinical features

SBP has a male : female ratio of 2 : 1, median age of 55 years, and primarily affects the axial skeleton, especially the vertebrae (Table 2) [3]. Malignant bone tumours of the spine are extremely rare (less than 0.05% of primary neoplasms). Solitary plasmacytoma is the most common separate entity within this group, accounting for about 30% of the total [4]. These tumours occur in the spine twice as often as other bony sites [5]. The most common symptom is pain, but it can also present with cord

Diagnostic criteria

Recommended diagnostic criteria are summarised in Table 3. On the basis of the data discussed above, the following criteria are recommended: (1) single area of bone destruction due to clonal plasma cells; (2) histologically normal marrow aspirate and trephine; (3) normal results on skeletal survey, including radiology of long bones; (4) no anaemia, hypercalcaemia or renal impairment due to plasma cell dyscrasia; (5) absent or low serum or urinary level of monoclonal immunoglobulin (level of >20 

Radiotherapy

Radiotherapy for the treatment of solitary plasmacytoma of bone has recently been reviewed [19]. Radical radiotherapy is the treatment of choice for SPB. In common with other rare tumours, the evidence base for treatment is largely composed of retrospective studies of small numbers of patients. Progression to multiple myeloma is common, despite high local control rates of 83–96% achieved with moderate doses of radiotherapy 12, 13, 14, 16, 20.

Data on dose–response relationships are weak in most

Management of apparent SBP with positive MRI

Patients presenting as SBP but found on MRI scan to have more extensive disease (i.e. marrow involvement) should be considered as having MM. However, if the plasmacytoma is the only cause of clinical symptoms and there is no other organ involvement, it would be appropriate to treat the plasmacytoma according to the guidelines above and to defer chemotherapy until there are signs of progression, as per the UK Myeloma Forum/British Committee for Standards in Haematology guidelines for

Context

Solitary extramedullary (soft tissue) plasmacytomas (SEP) are less common than SBP but carry a better prognosis, as most people can be cured by local radiotherapy [34]. Although SEP can arise throughout the body, almost 90% arise in the head and neck, especially in the upper respiratory tract, including the nasal cavity, sinuses, oropharynx, salivary glands and larynx 35, 36, 37, 38, 39. The next most frequent site is the gastrointestinal tract. A variety of other sites can rarely be involved,

Diagnostic criteria

CT or MRI is required to delineate the extent of the lesion, but the role of MRI of other areas in the staging of SEP has not been evaluated. As there is a low risk of progression to MM in these patients, and the role of MRI in the staging of SEP has not been studied, we do not consider MRI of the spine to be necessary for the diagnosis of SEP. Recommended diagnostic criteria are shown in Table 3.

Pathology review

For most patients, the diagnosis can be established by fine-needle aspiration or biopsy [51]. As

Radiotherapy

SEP are highly radiosensitive tumours. Local control rates of 80–100% are consistently reported with moderate doses of radiotherapy 14, 16, 20, 22. Radiotherapy dose and anatomical volume should be chosen to minimise early and late side-effects and maximise local control. There is no firmly established dose–response relationship, because of small patient series and low local failure rates. Tsang et al. [13] achieved local control in 13 out of 14 (93%) patients with 35 Gy. The only failure was in

Patient information and support

Provision of appropriate patient information and support forms an important part of the care of patients with SBP and SEP. General principles are the same as those for patients with myeloma [2]. The International Myeloma Foundation (UK) produces a booklet for patients with solitary plasmacytoma.

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 for Haematology and the publishers do not accept any legal responsibility or liability for any errors or omissions that may have been made.

Acknowledgements

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

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

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    ☆

    These Guidelines have been published in Clinical Oncology with the permission of Dr Steve Schey, Chairman of the UK Myeloma Forum.

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