Background. Primary nasal lymphoma is a rare disease. Although most pa
tients are initially seen in early Ann Arbor stages, their prognosis i
s poor. The prognostic significance of local tumor bulk has not been w
ell studied. Methods. Twenty-one patients with nasal lymphoma treated
between 1985 and 1992 were retrospectively studied. Sixteen patients (
76%) below the age of 75 years received combined radiotherapy and chem
otherapy. One young patient with early disease and 4 elderly patients
had radiotherapy alone. Twelve cases (57%) were diagnosed as pleomorph
ic T-cell lymphoma based on typical histologic features alone. Immunop
henotyping was performed in 10 cases; 8 were T cell and 2 were B cell.
Seventeen patients (81%) had Ann Arbor clinical stage IE disease, and
4 had stage IIE disease. The local tumor extent was assessed by endos
copy in all patients and by computerized tomography (CT) in 14 patient
s. Eleven local tumors (52%) extended to the posterior ethmoids, sphen
oid sinus, orbit, or beyond. Using a T-stage system, the prognostic si
gnificance of local tumor bulk was evaluated for stage IE patients. Re
sults. At a median follow-up time of 16.8 months, the lymphoma recurre
d in 13 patients; 10 patients had systemic relapse and 10 patients, lo
cal relapse. The 5-year actuarial overall sur vival rate was 24%. Comp
lete response to chemotherapy was achieved in 5 of 16 patients (31%).
Four of the 6 patients who remained alive and disease-free were chemot
herapy complete responders. Among stage IE patients, those with early
and those with advanced local disease did not have significantly diffe
rent survival. Conclusions. In view of the high systemic and local rel
apse rates, more-effective chemotherapy is needed to improve the survi
val rates, and the role of combined chemotherapy and radiotherapy shou
ld be evaluated. Further studies are required to identify patients at
high risk of relapse for clinical trials with investigational treatmen
t. (C) 1997 John Wiley & Sons, Inc.