13.          Non-Hodgkin’s Lymphoma, extranodal primaries

13.3        Primay mediastinal B-cell lymphoma (PMBL)

PMBL is a rapidly proliferating aggressive tumor which arises in the thymus and mainly affects young adults. Conventional or dose-intensified, but nonmyeloablative anthracycline-containing chemotherapy (e.g. CHOP, CEOP-Bleo. VACOP-B or MACOP-B) is generally accepted as the initial management, which may be followed by consolidation (radiation or high-dose chemotherapy with stem cell support) in patients with a high risk for recurrence (e.g. those with pleural or pericardial effusions). High-dose chemotherapy with autologous transplantation has been successful in patients with recurrent/refractory disease.

Literature: for review

            AVILES et al, Ann. Hematol. 81 (2002): 368-373 (CEOP-Bleo)

CAIROLI et al, Bone Marrow Transplant. 29 (2002): 473-477 (VACOP-B with early intensification integrating chemotherapy, autologous stem cell transplantation and radiotherapy)

            Van BESIEN et al, J, Clin. Oncol. 19 (2001): 1855 – 1864 (review)

            ZINZANI et al, Haematologica 86 (2001) : 187-191 (MACOP-B)