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Within diffuse large B-cell lymphomas, the Primary Mediastinal Large B-Cell Lymphoma has to be considered as a separate and well-defined clinico-pathological entity. Its tendency to target young adults makes its social impact particularly significant; hence, the General Practitioner carries the responsibility for an early diagnosis. On the contrary, the extreme complexity of the available therapies makes a quick referral to specialized Clinical Centres of outmost importance, since this remains the best way to enrol as many patients as possible in therapeutic protocols. Nowadays, good clinical results and a favourable outcome are achievable, but some questions remain open. The role of radiotherapy still has to be clarified, both as a complete remission consolidation, as well as a treatment of the residual disease. Conversely, a golden standard for the second line treatment has not been clearly established.

Cardiac onset of a diffuse large B-cell lymphoma (DLBCL) in a HIV+ patient with hypertrophic obstructive cardiomyopathy. The sequence of diagnosis, therapeutical planning and final outcome underlines the importance of an early diagnosis that cannot be conceived without an increased attention in this clinical setting (aspecific symptoms in an HIV positive subject) by the General Practitioner.

Primary central nervous system lymphoma is a quite infrequent disease within lymphoproliferative disorders. Moreover, it must be taken into account that, even considering the progressive spreading of ameliorated diagnostic tools and skills, histology remains the hinge of a correct diagnosis (stereotactic cerebral biopsy). Hence, randomised clinical protocols with a sufficiently large number of patients enrolled are difficult to perform and a generalized consensus on the best therapeutic options is hard to achieve. In this article, the more frequent practical and clinical aspects regarding diagnosis and staging of this rare disease are discussed. Furthermore, we analyze the different therapeutic options that were considered in a relatively recent past in which the median survival from diagnosis was not longer than a year, working down the pathway to the present and, finally, to the near future, always considering both first line and salvage therapy.The goal remains to optimize and standardize the therapeutic approach in order to achieve a generalized consensus that should lead to better overall clinical results.