title: Primary Mediastinal B-cell lymphoma with sclerosis: clinical and therapeutic evaluation of 22 patients.
abstract: In the last decade, there have been several reports on what is now recognized as a new clinical and pathological entity termed primarily mediastinal B-cell lymphoma (PMBCL) with sclerosis. This lymphoma presents unique clinical characteristics with an aggressive outcome and, at present, the best approach seems to be a combination of chemotherapy and radiotherapy. Between June 1989 and September 1994, twenty-two previously untreated patients with PMBCL with sclerosis were treated with a combination of third-generation chemotherapy regimen (MACOP-B or F-MACHOP) and mediastinal irradiation. All the patients presented with bulky mediastinal involvement; the radiologic clinical stage with evaluation of tumor size included computed tomography and Gallium-67-citrate SPECT. Twenty-one patients (95%) achieved a complete response and only one was resistant to treatment. Regarding 67Ga SPECT, 6 patients, including the nonresponder, showed persistent abnormal 67Ga uptake after chemotherapy; however after the mediastinal radiotherapy, all the patients except for the nonresponder were 67Ga-negative. The overall survival was 87%, with a median follow-up of 24 months from the time of diagnosis. Two of the patients who achieved complete response relapsed 7 and 10 months after completion of treatment, respectively. The relapse-free survival rate was 89% at 62 months (median 20 months). In patients presenting with bulky mediastinal PMBCL with sclerosis combined modality treatment using third-generation chemotherapy regimens and radiotherapy induces a good remission rate with greater than 80% chance of surviving disease-free, at 2 years. A longer follow-up before definitive conclusions are drawn is still warranted.
authors.affiliation: Institute of Hematology Seràgnoli, University of Bologna, Italy.
authors.forename: P L
authors.initials: PL
authors.lastname: Zinzani
authors.forename: M
authors.initials: M
authors.lastname: Bendandi
authors.forename: G
authors.initials: G
authors.lastname: Frezza
authors.forename: F
authors.initials: F
authors.lastname: Gherlinzoni
authors.forename: E
authors.initials: E
authors.lastname: Merla
authors.forename: M
authors.initials: M
authors.lastname: Salvucci
authors.forename: M
authors.initials: M
authors.lastname: Magagnoli
authors.forename: L
authors.initials: L
authors.lastname: Babini
authors.forename: S
authors.initials: S
authors.lastname: Tura
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001761:Bleomycin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005069:Evaluation Studies as Topic; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D005710:Gallium Radioisotopes; D006801:Humans; D002955:Leucovorin / Q000008:administration & dosage; D016393:Lymphoma, B-Cell / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D008297:Male; D008479:Mediastinal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D008727:Methotrexate / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D012598:Sclerosis; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D005710:Gallium Radioisotopes; D003561:Cytarabine; D001761:Bleomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D002955:Leucovorin; D005472:Fluorouracil; D011241:Prednisone; D008727:Methotrexate