Primary mediastinal large B-cell lymphoma with sclerosis: a clinical study of 89 patients treated with MACOP-B chemotherapy and radiation therapy.
OBJECTIVE
Primary mediastinal large B-cell lymphoma (PMLBCL) with sclerosis has recently been recognized as a specific clinical and pathologic entity for which the best therapeutic approach seems to be a combination of chemotherapy and radiotherapy.


METHODS
Between 1989 and 1998, 89 previously untreated patients with PMLBCL with sclerosis were treated with a combination of a third-generation chemotherapy regimen (MACOP-B) and mediastinal radiation therapy. The response evaluations were examined after chemotherapy and at the end of radiotherapy.


RESULTS
Twenty-three (26%) patients achieved a complete response (CR) and 59 (66%) obtained a partial response (PR) after the MACOP-B regimen. After radiation therapy, 55/59 (93%) of the patients in PR achieved CR. The CR rate at the end of the treatment was 88% (78/89). Only 7 (8%) patients were non-responders. Among the 78 patients who obtained a CR there were 7 (9%) relapses in a median follow-up of 5 months (all relapses occurred within 9 months); the other 71 patients are currently in continuous CR with a median follow-upof 45 months (range, 4-110 months). Projected overall survival was 86% at 9 years; the relapse-free survival curve of the 78 patients who achieved CR was 91% at 9 years.


CONCLUSIONS
In patients with PMLBCL with sclerosis, combined modality treatment using the MACOP-B chemotherapy regimen and radiation therapy induces a good remission rate with the patients having a greater than 90% chance of surviving disease-free at 9 years. Radiotherapy often plays a pivotal role in obtaining CR status.
Istituto di Ematologia e Oncologia Medica Seràgnoli, Policlinico S. Orsola, via Massarenti 9, 40138 Bologna Italy. plzinzo@med.unibo.it
P L
PL
Zinzani
M
M
Martelli
M
M
Bendandi
A
A
De Renzo
A
A
Zaccaria
E
E
Pavone
M
M
Bocchia
B
B
Falini
M
M
Gobbi
F
F
Gherlinzoni
V
V
Stefoni
M
M
Tani
S
S
Tura
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000592:standards / Q000627:therapeutic use; D001761:Bleomycin / Q000008:administration & dosage / Q000592:standards; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000592:standards; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage / Q000592:standards; D005260:Female; D006801:Humans; D002955:Leucovorin / Q000008:administration & dosage / Q000592:standards; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D008297:Male; D008479:Mediastinal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D008727:Methotrexate / Q000008:administration & dosage / Q000592:standards; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage / Q000592:standards; D012598:Sclerosis; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage / Q000592:standards
D001761:Bleomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D002955:Leucovorin; D011241:Prednisone; D008727:Methotrexate