Limited data are available concerning treatment and outcome of primary lymp
homa of the breast (PLB), especially after CHOP (cyclophosphamide, hydroxyd
oxorubicin, vincristine, prednisone) chemotherapy. We retrospectively revie
wed 20 consecutive cases of localized PLB seen at our institution over a 20
year period. All PLB were of B-cell origin: treatment was CHOP or a CHOP-n
e regimen in all patients. Sixteen of the 20 patients achieved complete rem
ission (CR) and two achieved partial remission (>75% tumour regression). Tw
o patients had progressive disease on therapy. With a median follow-up of 5
4 months, six patients relapsed after 8-66 months. Two of the relapses invo
lved the central nervous system (CNS) (isolated in one case, associated wit
h other sites of relapse in the other). The two patients who achieved parti
al remission also had progression in the CNS, 4 and 8 months after the end
of CHOP chemotherapy. All four patients have died as a result of their dise
ase 3, 6, 10 and 13 months after CNS relapse. Of the 16 centroblastic diffu
se large B-cell lymphoma (DLCL), three had CNS disease at relapse. We also
observed three (15%) controlateral breast relapses. Thirteen of the initial
20 patients are alive in CR, six patients have died as a result of their l
ymphoma and one of unrelated disease. In conclusion, we observed a high inc
idence of CNS relapse in this group of localized extranodal lymphoma, stron
gly suggesting that CNS prophylaxis should be associated with systemic chem
otherapy in localized PLB.