Canterbury DHB

Context

Extra Medullary Plasmacytoma

This is more likely to be truly localised than plasmacytoma of bone. The most common sites are in the upper respiratory tract and GI tract. Careful staging is essential. 25% of patients will have a monoclonal protein.

This should be distinguished from reactive plasma cells or lymphoma.

It should be demonstrated that the infiltrate consists entirely of plasma cells and that there is no B cell component. In this regard CD138, MUM1/IRF4, CD20 and PAX5 are the most useful markers although it should be recognised that CD20 and PAX5 are sometimes expressed in plasma cell malignancies. Monoclonality and /or an aberrant plasma cell phenotype should be demonstrated with useful markers being CD19, CD56, CD27, CD117 and cyclin D1.

Local radiotherapy 40-50Gy is the treatment of choice. 70% of such patients will be alive and disease-free at 10 years.

Treatment by radical radiotherapy should encompass the primary tumour with a margin of at least 2 cm. The cervical nodes should be included if involved. The first echelon cervical nodes should be included in EP of Waldeyer’s ring. For EP up to 5 cm a radiotherapy dose of 40 Gy in 20 fractions is recommended. For EP >5 cm a higher dose of up to 50 Gy in 25 fractions is recommended.

See BCSH Guidelines 2014.

About this Canterbury DHB document (5612):

Document Owner:

Sean Macpherson (see Who's Who)

Issue Date:

September 2014

Next Review:

September 2016

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 5612