Extramedullary plasmacytoma SEP

Diagnostic criteria

• Generally no paraprotein in serum or urine though small band may be present.

• Extramedullary tumour of clonal plasma cells.

• BM not consistent with MM.

• Normal skeletal survey (and MRI of spine and pelvis).

• No myeloma-related organ or tissue impairment (end-organ damage).

• Rare; may occur anywhere but 90% in head and neck; most in upper airways.

• Diagnosis requires biopsy or FNA of the lesion; exclusion of MM and other lesions.

• <25% have serum or urine paraprotein.

• Treat with radical radiotherapy (40Gy; 50Gy if lesion >5cm) including cervical lymph nodes when involved; radical surgery only for SEP outside head and neck.

• Most cured; <5% local recurrence; relapse <30%; MM, SBP or soft tissue involvement: chemotherapy for refractory or relapsed disease

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