The current therapy of choice is prednisone and melphalan, although improvement with this regimen has been only anecdotal. Currently, a trial of chemotherapy is felt to be warranted based on anecdotal evidence. Since the vast majority of lesions of the head and neck, with the exception of macroglossia, represent localized disease, therapy consisting of surgical excision has been far more successful. In the larynx, a conservative approach has been recommended consisting of local excision using laser or microlaryngeal instruments with conservation of surrounding tissue. Recent literature has advocated CO2 laser as the treatment of choice.
Recurrence is rare, and, if it occurs, can be treated with further conservative excision.
Partial glossectomy for macroglossia represents localized surgery for a systemic disease. Results of partial glossectomy have been mixed but are generally favorable. Goals are to restore speech and swallowing, decrease sialorrhea, and improve cosmetic appearance. Prognosis in patients with systemic amyloidosis is poor. Almost all patients with light chain disease are dead within three years of diagnosis.