What is primary central nervous system lymphoma (PCNSL)?

Updated: Jul 06, 2021
  • Author: Caroline T Goldin, MD; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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Primary central nervous system lymphoma (PCNSL) has been known by many other names, including reticulum cell sarcoma, diffuse histiocytic lymphoma, and microglioma. The proliferation of names reflects initial uncertainty about the cell of origin.

PCNSL is now known to be a form of extranodal, high-grade non-Hodgkin B-cell neoplasm, usually large cell or immunoblastic type. It originates in the brain, leptomeninges, spinal cord, or eyes; typically remains confined to the CNS; and rarely spreads outside the nervous system. Therefore, it can be classified as stage 2 disease.

Previously a rare tumor accounting for less than 2% of cerebral neoplasms, PCNSL is being seen with increasing frequency in immunocompetent patients.

Although the cells of origin are lymphocytes, PCNSL should be considered a brain tumor, because its therapeutic challenges resemble those of other brain tumors. In particular, drug delivery is impaired by the blood-brain barrier, and cerebral toxicity limits the use of available treatment modalities.

Most PCNSLs (about 90%) are diffuse large B-cell lymphomas (DLBCLs); the remaining 10% are poorly characterized low-grade lymphomas, Burkitt lymphomas, and T-cell lymphomas. [1]

Primary symptoms may result from local mass effect due to raised intracranial pressure, from ocular involvement, or from focal deposits on cranial or spinal nerve roots.

Also see Non-Hodgkin Lymphoma.

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