Extramedullary plasmacytoma - Tumor occurrence and therapeutic concepts

BACKGROUND, Extramedullary plasmacytoma (EMP) is a rare entity belonging to the category of non-Hodgkin lymphoma. EMPs make up 4% of all plasma cell t umors and occur mainly in the upper aerodigestive tract (UAD). Seven patien ts with EMP included in this evaluation were under the authors' care and ha ve been clinically followed since 1990. Because there are no general guidel ines for the treatment of patients with EMP, the authors tried to obtain de tailed data about the occurrence of this disease and also reviewed the ther apies that have been used. To do so, they evaluated all EMP cases published in the medical literature until now and included their own experience. METHODS. Based on the clinical course and follow-up of their own EMP patien ts, the authors evaluated and reinvestigated all EMP cases cited in MEDLINE , Index Medicus, DIMDI (Deutsches Institut fur medizinische Dokumentation u nd Information, Cologne, Germany), and the reference lists of the publicati ons found through these sources. RESULTS. In a detailed literature search, more than 400 publications betwee n 1905 and 1997 were found, and these revealed that EMP mainly occurs betwe en the fourth and seventh decades of life. Seven hundred fourteen cases (82 .2%) were found in the UAD, and 155 cases (17.8%) were found in other body regions. The following therapeutic strategies were used to treat patients w ith EMP of the UAD: radiation therapy alone in 44.3%, combined therapy (sur gery and radiation) in 26.9%, and surgery alone in 21.9%. The median overal l survival or recurrence free survival was longer than 300 months for patie nts who underwent combined intervention (surgery and radiation). This resul t was statistically highly significant (P = 0.0027, log rank test) compared with the results for patients who underwent surgical intervention alone (m edian survival time, 156 months) or radiation therapy alone (median surviva l time, 144 months). In most cases of non-UAD EMP, surgery was performed (s urgery alone, 55.6%; surgery and radiation combined, 19.8%; radiation alone , 11.1%), but there were no statistical differences in survival (P = 0.62). Overall, after treatment for EMP in the UAD, 61.1% of all patients had no recurrence or conversion to systemic involvement (i.e., multiple myeloma, M M); however, 22.0% had recurrence of EMP, and 16.1% had conversion to MM. A fter treatment for EMP in non-UAD areas, 64.7% of all patients had no recur rence or MM, 21.2% had recurrence, and 14.1% had conversion to MM. CONCLUSIONS. The current investigation provides evidence that surgery alone gives the best results in cases of EMP of the UAD when resectability is go od. However, if complete surgical tumor resection is doubtful or impossible and/or if lymph node areas are affected, then combined therapy (surgery an d radiation) is recommended. These results, which were obtained from retros pective studies, should be confirmed in randomized trials comparing surgery with combined radiation therapy and surgery. Cancer 1999;85:2305-14. (C) 1 999 American Cancer Society.