MYD88 L265P mutation in primary central nervous system lymphoma is associated with better survival: A single-center experience.

Autor:	Curran OE; Cellular Pathology, University Hospital of Wales, Cardiff CF14 4XW, UK.; Neuropathology Unit, Department of Pathology, Western General Hospital, Edinburgh EH4 2XU, UK., Poon MTC; Usher Institute, University of Edinburgh, Edinburgh EH16 4UX, UK.; Department of Clinical Neurosciences, Royal Infirmary of Edinburgh, Edinburgh EH16 4SA, UK., Gilroy L; Molecular Pathology, Western General Hospital, Edinburgh EH4 2XU, UK., Torgersen A; Neuropathology Unit, Department of Pathology, Western General Hospital, Edinburgh EH4 2XU, UK., Smith C; Neuropathology Unit, Department of Pathology, Western General Hospital, Edinburgh EH4 2XU, UK., Al-Qsous W; Department of Pathology, Western General Hospital, Edinburgh EH4 2XU, UK.
Jazyk:	angličtina
Zdroj:	Neuro-oncology advances [Neurooncol Adv] 2021 Jul 07; Vol. 3 (1), pp. vdab090. Date of Electronic Publication: 2021 Jul 07 (Print Publication: 2021).
DOI:	10.1093/noajnl/vdab090
Abstrakt:	Background: The myeloid differentiation primary response gene ( MYD88 ) mutation in primary central nervous system lymphomas (PCNSL) may be associated with unfavorable prognosis; however, current evidence remains limited. We aimed to characterize PCNSLs by integration of clinicopathological, molecular, treatment, and survival data. Methods: We retrospectively identified and validated 57 consecutive patients with PCNSLs according to the 2017 WHO classification of lymphoid neoplasms over 13 years. Formalin-fixed paraffin-embedded tumor samples underwent polymerase chain reaction assay to detect MYD88 mutation. We used Cox regression for survival analysis, including age, treatment, and MYD88 as covariates. We searched the literature for studies reporting demographics, treatment, MYD88 , and survival of PCNSL patients and incorporated individual patient data into our analyses. Results: The median age was 66 years and 56% were women. All 57 patients had PCNSL of non-germinal center cell subtype and the majority (81%) received either single or combined therapies. There were 46 deaths observed over the median follow-up of 10 months. MYD88 mutation status was available in 41 patients of which 36 (88%) were mutated. There was an association between MYD88 mutation and better survival in the multivariable model (hazard ratio [HR] 0.277; 95% confidence interval [CI]: 0.09-0.83; P = .023) but not in a univariable model. After incorporating additional 18 patients from the literature, this association was reproducible (HR 0.245; 95% CI: 0.09-0.64; P = .004). Conclusions: Adjusting for confounders, MYD88 -mutant PCNSL appears to show improved survival. While further validation is warranted, detection of MYD88 mutation will aid the identification of patients who may benefit from novel targeted therapies. (© The Author(s) 2021. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)
Databáze:	MEDLINE
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