Mediastinal B-cell lymphoma with sclerosis: clinical features and treatment results in 10 patients

Mediastinal large-B-cell lymphoma with sclerosis is now considered to be a discrete subtype of lymphoma. It probably originates in the thymus, a T-cell organ. Early publications consider this lymphoma as an aggressive disorder with poor prognosis. We studied retrospectively ten consecutive patients with mediastinal B-cell lymphoma with sclerosis seen in the department of hematology. Nine were women. The median age at diagnosis was 38.3 years (16-60). Dyspnea (experienced by 7 patients), chest pain (5) and cough (10) were the most common clinical features at presentation. Superior vena cava syndrome occurred in three patients. Five had infiltration of the chest wall or of the pulmonary tissue. Four patients were in clinical stage I (all bulky > 10 cm), four in stage IIE, one was in stage IIE and one in stage IV (Ann Arbor classification). All patients were treated with intensive chemotherapy, mostly containing cyclophosphamide, doxorubicin, vincristine or vindesine, bleomycin and prednisone, combined with etoposide or teniposide and methotrexate. Nine patients responded well to chemotherapy (tumor reduction > 75%). One patient progressed. Eight patients received involved field radiotherapy (36-40 Gy) after chemotherapy. The two other patients were treated with intensive chemotherapy (BEAC, BCNU, etoposide, cytarabine, cyclophosphamide), followed by autologous bone marrow transplantation. Two patients died: one patient received autologous bone marrow transplantation in partial remission and relapsed after 6 months; the other patient had progressive disease despite chemotherapy, surgery and radiotherapy. Mean follow-up is 54.6 months (15-118) with 8 patients still remaining in complete remission. In patients with mediastinal B-cell lymphoma, tumour localisation is often limited to the thorax.(ABSTRACT TRUNCATED AT 250 WORDS)