PMID- 34927072 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211221 IS - 2632-010X (Electronic) IS - 2632-010X (Linking) VI - 14 DP - 2021 Jan-Dec TI - An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage. PG - 2632010X211065692 LID - 10.1177/2632010X211065692 [doi] LID - 2632010X211065692 AB - Extranodal NK/T-cell lymphoma (ENKTL) is a well-defined cytotoxic lymphoma strongly associated with Epstein-Barr virus (EBV) infection, commonly affecting the nasopharynx and upper aerodigestive tract. Primary central nervous system (CNS) involvement is rare, and only 17 cases were previously reported in the literature. Here, we report the case of a 44-year-old male admitted with a 3-month history of personality changes and progressive right leg weakness. Brain magnetic resonance imaging studies (MRIs) revealed multiple rim-enhancing brain lesions bilaterally. An extensive clinical and laboratory workup was unrevealing, and 2 brain biopsies were initially considered inconclusive. Pertinently, no systemic lymphoproliferative disorder was identified. The patient initially experienced remarkable clinical improvement with dexamethasone, pulse methylprednisolone, and rituximab therapy. However, he eventually had rapid clinical deterioration, was found to have increased brain lesions, and died nearly 6 months after the initial presentation. During this time, the second brain biopsy was found to show involvement by T-cell lymphoma of NK-cell lineage, which was EBV negative. No post-mortem examination was done to identify any systemic lymphoma. This case serves to expand the spectrum of lymphomas involving the CNS. CI - (c) The Author(s) 2021. FAU - Kajtazi, Naim I AU - Kajtazi NI AUID- ORCID: 0000-0002-9983-3584 AD - Department of Neurology, National Neurosciences Institute, King Fahad Medical City, Riyadh, KSA. FAU - Bafaquh, Mohammed AU - Bafaquh M AD - Department of Neurosurgery, National Neurosciences Institute, King Fahad Medical City, Riyadh, KSA. FAU - Ghamdi, Juman Al AU - Ghamdi JA AD - Medical Imaging Administration, Neurointervention Radiology, King Fahad Medical City, Riyadh, KSA. FAU - AlEissa, Zahra AU - AlEissa Z AD - Department of Neurology, National Neurosciences Institute, King Fahad Medical City, Riyadh, KSA. FAU - Shmeikh, Arwa Al AU - Shmeikh AA AD - Department of Neurosurgery, National Neurosciences Institute, King Fahad Medical City, Riyadh, KSA. FAU - Alsaeed, Ali AU - Alsaeed A AD - Internal Medicine Department, Infectious Diseases, King Fahad Medical City, Riyadh, KSA. FAU - Sulaiman, Tarek AU - Sulaiman T AD - Internal Medicine Department, Infectious Diseases, King Fahad Medical City, Riyadh, KSA. FAU - Vizcaino, M Adelita AU - Vizcaino MA AD - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA. FAU - Al Hameed, Majed AU - Al Hameed M AD - Department of Neurology, National Neurosciences Institute, King Fahad Medical City, Riyadh, KSA. FAU - Raghunathan, Aditya AU - Raghunathan A AD - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA. LA - eng PT - Journal Article DEP - 20211212 PL - United States TA - Clin Pathol JT - Clinical pathology (Thousand Oaks, Ventura County, Calif.) JID - 101741182 PMC - PMC8671822 OTO - NOTNLM OT - Epstein-Barr virus (EBV) infection OT - NK (natural killer) cells OT - Primary CNS lymphoma OT - extranodal NK/T-cell lymphoma (ENKTL) COIS- Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2021/12/21 06:00 MHDA- 2021/12/21 06:01 PMCR- 2021/12/12 CRDT- 2021/12/20 06:26 PHST- 2021/09/12 00:00 [received] PHST- 2021/11/01 00:00 [accepted] PHST- 2021/12/20 06:26 [entrez] PHST- 2021/12/21 06:00 [pubmed] PHST- 2021/12/21 06:01 [medline] PHST- 2021/12/12 00:00 [pmc-release] AID - 10.1177_2632010X211065692 [pii] AID - 10.1177/2632010X211065692 [doi] PST - epublish SO - Clin Pathol. 2021 Dec 12;14:2632010X211065692. doi: 10.1177/2632010X211065692. eCollection 2021 Jan-Dec.