Monoclonal extramedullary plasmacytoma (EMP) is a rare, low-grade lymphoma
found predominantly in the head and neck region. Only since the introductio
n of immunophenotyping techniques 2 decades ago has it been possible to dif
ferentiate EMP from benign polyclonal plasma cell proliferation. The purpos
e of this study was to trace the evolutionary profile of the disease under
consideration of monoclonality assessment. The records of 24 patients with
morphologically diagnosed EMP treated in a single institution underwent cli
nical and pathological review. Only 14 patients had true monoclonal plasmac
ytoma. No EMP-related deaths occurred. Two patients had local recurrence, a
nd 2 patients developed multiple myeloma. Review of the literature confirms
the low-grade malignancy of EMP. Diagnostic procedures must exclude benign
polyclonal plasmacytoma, multiple myeloma, and solitary hone plasmacytoma.
The slow natural progression of the disease and the rarity of secondary mu
ltiple myeloma favor nonmutilating local surgery whenever possible to avoid
the long-term sequelae of radiotherapy.