Primary CNS lymphoma: a clinician’s guide

Abstract

Primary CNS lymphoma is a high malignant disease of the brain which can lead rapidly to death if diagnosis and/or the start of treatment is delayed. The age at time of diagnosis is a strong factor influencing prognosis so that in younger patients <65 years of age long-term survival may be achieved in a substantial percentage of patients, while in elderly patients long-term survival is seen much more rarely. First-line therapy consists of high-dose methotrexate-based (poly)chemotherapy. This review provides an overview of clinical presentation, steps to diagnosis, detailed information about current treatment concepts and specific information for particular clinical situations.

Keywords::

• autologous stem cell transplantation
• HD-MTX
• intraventricular therapy
• lymphoma
• neurotoxicity
• PCNSL
• polychemotherapy

Acknowledgements

With the permission of Kohlhammer Publishers (Stuttgart, Germany), translations of the following German handbook chapter were used as a template in this article: Glas M, Birnbaum T, Herrlinger U. Primäre ZNS-lymphome und ZNS-manifestationen bei systemischen lymphomen. In: Therapie und Verlauf Neurologischer Erkrankungen (6th Edition). Brandt T, Diener HC, Gerloff C (Eds). Kohlhammer Publishers, Stuttgart, Germany (2012). The authors thank K Kuchelmeister (Institute of Neuropathology, University Hospital of Bonn, Germany) for providing histological images and H Urbach (Head of Departement of Neuroradiology, University Hospital of Bonn, Bonn, Germany) for providing MRIs.

Financial & competing interests disclosure

U Herrlinger and M Glas have received honoraria from Mundipharma (manufacturer of DepoCyte^®) in the past. U Herrlinger has also received honoraria from Schering-Plough (now MSD), manufacturer of temozolomide and Medac, manufacturer of temozolomide and CCNU. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.