Extramedullary plasmacytomas in the head and neck region.
Extramedullary plasmacytoma (EMP) arises outside the bone marrow, particularly in the head and neck region (nasopharynx, nose cavity, sinuses, and tonsils), and can be associated with multiple myeloma (MM). Three cases of EMP in the head and neck region are described: a first case describes an EMP of the subglottis 3 years after treatment of MM, a second case of an EMP solitary in the middle ear presenting as a jugular foramen syndrome, and a third case of an EMP localised at the epiglottis, recurring at the floor of the nose cavity. Treatment of each EMP was surgical. We reviewed literature about aetiology, clinical course, diagnostics, treatment and prognosis. Important presenting symptoms vary from epistaxis, rhinorrhoea, a sore throat, dysphonia to haemoptoea. Association with MM must be confirmed or excluded. Histopathological examination, with immunological staining or flow cytometry confirms the diagnosis. CT and MRI are useful in staging EMP. The treatment of EMP is surgery and/or radiotherapy. The prognosis depends on tumour size (>5 cm) and nodal involvement. The 10-year survival rate is 50-80%.
Straetmans
Jos
J
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, P. Debyelaan 25, P.O Box 5800, 6202 AZ, Maastricht, The Netherlands. jstra@skno.azm.nl
Stokroos
Robert
R
D000368:Aged; D005158:Facial Paralysis / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006258:Head and Neck Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007828:Laryngoscopy; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D009367:Neoplasm Staging; D010954:Plasmacytoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D023981:Sarcoma, Myeloid / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed