Primary laryngeal lymphoma is a very rare entity, with fewer than 50 c
ases reported in the English literature in the past 60 years. Close sc
rutiny of some of these case reports reveals that the larynx was not a
lways the only site of involvement, thereby diminishing the total numb
er of patients with primary laryngeal lymphoma to fewer than 35. The a
uthors report a series of six patients, who were seen and evaluated at
the Mayo Clinic between 1952 and 1995, with stage IAE non-Hodgkin's l
ymphoma of the larynx. Three patients had large-cell lymphomas accordi
ng to the REAL (Revised European-American Lymphoid) classification. Th
e other three had a small lymphocytic lymphoma, follicular small cleav
ed lymphoma, and follicular mixed lymphoma. All patients received radi
ation therapy alone as initial therapy for their disease and all patie
nts had a complete remission to initial therapy. Four patients subsequ
ently relapsed and the histology at relapse was the same as the initia
l histology in all four patients. Five patients have died, three of ly
mphoma, with a median survival of 67 months (range, 40 to 228 months).
In view of the heterogeneity of histologies in this group of lymphoma
s, the variability in duration of response, and the significant number
of patients who died of their disease, it is more likely that primary
laryngeal lymphoma is an unusual presentation of non-Hodgkin's lympho
ma than a separate disease entity. Despite the small number of patient
s in this study, the data would suggest that patients are best treated
according to the histology of the lymphoma, rather than the limited s
tage and location of the disease.