Practice guidelines for the management of extranodal non-Hodgkin's lymphomas of adult non-immunodeficient patients. Part I: primary lung and mediastinal lymphomas. A project of the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation.

Extranodal non-Hodgkin's lymphomas constitute 20-25% of overall non-Hodgkin's lymphomas cases and can be managed with very different therapeutic strategies. Therefore, the Italian Society of Hematology and the two affiliate societies (the Italian Society of Experimental Hematology and the Italian Group of Bone Marrow Transplantation) appointed a panel of experts to produce clinical practice-guidelines for the management of these conditions. Primary lung and mediastinal lymphomas were the objective of this part of the project. The panel of experts produced the following key recommendations that were graded according to the strength of evidence and clinical judgement. The first-line therapy for non-MALT primary lung non-Hodgkin's lymphomas should include anthracycline-based chemotherapy with CHOP or CHOP-like, MACOP-B or MACOP-B-like regimens (grade D). Rituximab association with chemotherapy needs to be evaluated within approved clinical trials. Second-line therapy with high-dose chemotherapy and autologous stem cell transplantation is recommended (grade B). In patients with MALT primary lung non-Hodgkin's lymphomas, the recommended first-line therapy should include chlorambucil, CHOP, CHOP-like or fludarabine-containing regimens (grade B). Radiotherapy is to be reserved for patients with a unique, small lesion in a poorly mobile site and with contraindication to surgery (grade D). Rituximab should be administered only within approved clinical trials. For treatment of primary mediastinal large B-cell lymphomas, the recommended first-line therapy is a chemotherapy and radiotherapy association (grade B). An anthracycline-based chemotherapy with CHOP, MACOP-B or VACOP-B is recommended (grade B). Rituximab combination with chemotherapy is highly suggested but only for patients enrolled into approved clinical trials. Patients with an inadequate early response should be candidates for early intensification with high-dose chemotherapy (grade C). Patients with refractory or relapsed disease should undergo rescue programs including intensive, non-cross-resistant debulking treatment followed, in chemosensitive patients, by high-dose chemotherapy and autologous stem cell transplantation (grade B).