What causes primary central nervous system lymphoma (PCNSL)?

Updated: Jan 19, 2018
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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The DLBCL type of PCNSL is composed of immunoblasts or centroblasts that have a predilection for blood vessels. Lymphoid clustering around small cerebral vessels is typically seen. Reactive T-cell infiltrates can also be present in varying degrees.

Evidence from a study by Sugita et al supported the concept that PCNSLs originate from a later germinal center to an early postgerminal center, and they may be capable of further maturation steps. [3] The study involved 32 PCNSL cases, with expression of proteins found at different stages in lymphocyte development; tumor specimens were immunophenotyped for antigens associated with germinal centers (CD10, Bcl-6) and with nongerminal center stages (SHP-1, CD138). In 30 of 32 cases, tumors were positive for SHP-1 but negative for CD138.

However, current evidence argues against the possibility that malignant lymphocytes perform further maturational steps. [4, 5] The tumor likely arises in an extraneural environment with subsequent localization to the CNS, possibly by virtue of a specific neurotropism.

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