Primary central nervous system lymphoma (PCNSL)

Primary central nervous system lymphoma (PCNSL) is a type of central nervous system restricted non-Hodgkin lymphoma, whose histopathological diagnosis is majorly large B cell lymphoma.

They are extranodal, of the diffuse large B cell type that is confined to the brain, eyes, leptomeninges, or spinal cord, in the absence of systemic lymphoma.

Primary Central Nervous System Lymphoma Classification.

Primary cerebral lymphoma represents 4%–7% of primary brain tumors, and its incidence has increased in the last 3 decades ¹⁾.

see Primary central nervous system lymphoma epidemiology.

see Primary central nervous system lymphoma in AIDS

History

Bailey first described PCNSL as “perithelial sarcoma” of the CNS and Henry in 1974 recognized its lymphoid origin ²⁾.

Generally, PCNSL of DLBCL type is a highly aggressive tumor and although its prognosis is poor, about one-third of younger patients can hope for cure of the disease ³⁾.

see Lymphomatosis cerebri.

It typically presents as multifocal T2 hyperintense lesions that both enhance after gadolinium administration and demonstrate mass effect. A case study in 1999 coined the term ‘lymphomatosis cerebri’ to describe an exceedingly rare variant of PCNSL characterised by diffuse parenchymal infiltration of lymphomatous cells.

Although exceedingly rare, primary central nervous system lymphoma can present as growing intracerebral hemorrhage due to repeated intratumoral hemorrhages. High expression of vascular endothelial growth factor and the mass effects of hemorrhage could be associated with the onset and growth of intracerebral hemorrhage. Early evaluation and meticulous observation are important to avoid progressive, life-threatening situations in such cases ⁴⁾.

see Primary central nervous system lymphoma diagnosis.

Primary central nervous system lymphoma differential diagnosis.

see Primary central nervous system lymphoma treatment.

Primary central nervous system lymphoma outcome.

see Primary central nervous system lymphoma case series.

Only three completed randomised trials are available for primary CNS lymphoma: one phase 3 and two phase 2 trials ⁵⁾ ⁶⁾ ⁷⁾.

Primary central nervous system lymphoma case reports.

¹⁾

Surawicz TS, McCarthy BJ, Kupelian V, et al. Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990–1994. Neuro-oncol 1999;1:14–25.

²⁾

Ney DE, Deangelis LM. Non- Hodgkin Lymphomas. Philadelphia: Lippincott Willilams & Wilkins; 2010. Management of central nervous system lymphoma; pp. 527–539.

³⁾

Juergens A, Pels H, Rogowski S, Fliessbach K, Glasmacher A, Engert A, Reiser M. et al. Long-term survival with favorable cognitive outcome after chemotherapy in primary central nervous system lymphoma. Ann Neurol. 2010;67(2):182–189. doi: 10.1002/ana.21824.

⁴⁾

Matsumoto Y, Kashimura H, Aso K, Saura H, Osakabe M, Kurose A. Primary Central Nervous System Lymphoma Presenting as Growing Intracerebral Hemorrhage. World Neurosurg. 2018 Aug;116:155-158. doi: 10.1016/j.wneu.2018.05.107. Epub 2018 May 23. PMID: 29803066.

⁵⁾

Ferreri AJ, Reni M, Foppoli M, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 2009; 374: 1512–20.

⁶⁾

Thiel E, Korfel A, Martus P, et al. High-dose methotrexate with or without whole brain radiotherapy for primary CNS lymphoma (G-PCNSL-SG-1): a phase 3, randomised, non-inferiority trial. Lancet Oncol 2010; 11: 1036–47.

⁷⁾

Omuro AM, Chinot O, Taillandier L, et al. Methotrexate and temozolomide versus methotreaxate, procarbazine, vincristine, and cytarabine for primary CNS lymphoma in the elderly population: an intergroup ANOCEF-GOELAMS randomised phase 2 trial. Lancet Haematol 2015; 2: e251–59.