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Case Report 

Cancer Research Frontiers. 2018; 4(1): 131-143. doi: 10.17980/2018.131

Atypical Presentation and Aggressive Evolution of Primary CNS Lymphoma (PCNSL)

Karan Topiwala1,2, Karan Tarasaria1,2, Jussie Lima1,2, Xianyuan Song2a, Ahmad Daher 2b, Amre Nouh2

University Of Connecticut School Of Medicine1, Hartford Hospital2, Department of Neurology, Department of Pathology2a, Department of Neuro-Oncology2b

 

*Corresponding author: Amre Nouh, Hartford Hospital, Department Of Neurology, 80 Seymour St., Hartford, CT 06106, USA. Tel: 860.972.3621. Email: Amre.Nouh@hhchealth.org

Citation: Karan Topiwala, et al. Atypical Presentation and Aggressive Evolution of Primary CNS Lymphoma (PCNSL). Cancer Research Frontiers. 2018; 4(1): 131-143. doi: 10.17980/2018.131

Copyright: @ 2018 Karan Topiwala, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Competing Interests: The authors declare no competing financial interests.

Received June 5, 2018; Revised July 30, 2018; Accepted Sept 4, 2018. Published Oct 10, 2018

 

Abstract

Patients with Primary CNS Lymphoma (PCNSL) typically present with focal neurological deficits with neuroimaging showing a solitary, non-haemorrhagic mass. We describe a 48-year-old-woman who presented with her first lifetime seizure with no enhancing intracranial mass, who 7 months later, developed focal deficits and was found to have multifocal enhancing lesions associated with intracerebral haemorrhage, which on biopsy were consistent with a PCNSL. This case highlights several atypical aspects of PCNSL, from its presentation with GTCS as seen in about 14% cases, to the atypical MRI finding of non-enhancing bi-temporal lobe hyperintensities at presentation with the subsequent evolution of multifocal haemorrhagic lesions. It also highlights the emerging genetic and molecular diagnostic markers in serum and CSF, while underscoring the importance of appropriately timing CSF evaluation for an accurate and reliable cytometric analysis. We also review the relevant literature on the imaging characteristics of PCNSL. Finally, it reviews some of the atypical cases of PCNSL published in the English literature and adds to that list as an aggressive symptomatic PCNSL that presented without an enhancing-intracranial mass-lesion, with the subsequent development of multifocal lesions associated with an intraparenchymal haemorrhage.

Keywords: CNS lymphoma, PCNSL, MRI, Intraparenchymal haemorrhage

 

 

 

 

 

 

 

 

 

 

 

 

 

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