ABSTRACT
INTRODUCTION The central nervous system (CNS) is affected by a wide variety of haematopoietic tumours. The term ‘lymphoma’ in the CNS comprises several entities that differ fundamentally in their clinical manifestation, course of disease, prognosis, morphology and underlying pathogenesis. They are strongly associated with specific clinical features, among which the immune status is of particular relevance. Careful examination to precisely classify lymphoma is a prerequisite for subsequent patient management requiring an interdisciplinary approach of neurologists, stereotactic neurosurgeons, haematologists, neuropathologists and molecular geneticists. Importantly, primary CNS lymphoma (PCNSL) has to be distinguished from systemic lymphoma secondarily spreading to the CNS, which may affect the CNS either exclusively or as part of multiorgan involvement.
