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Published in: Fluids and Barriers of the CNS 1/2017

Open Access 01-12-2017 | Research

Patterns of relapse in primary central nervous system lymphoma: inferences regarding the role of the neuro-vascular unit and monoclonal antibodies in treating occult CNS disease

Authors: Prakash Ambady, Rongwei Fu, Joao Prola Netto, Cymon Kersch, Jenny Firkins, Nancy D. Doolittle, Edward A. Neuwelt

Published in: Fluids and Barriers of the CNS | Issue 1/2017

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Abstract

Background and purpose

The radiologic features and patterns of primary central nervous system lymphoma (PCNSL) at initial presentation are well described. High response rates can be achieved with first-line high-dose methotrexate (HD-MTX) based regimens, yet many relapse within 2 years of diagnosis. We describe the pattern of relapse and review the potential mechanisms involved in relapse.

Methods

We identified 78 consecutive patients who attained complete radiographic response (CR) during or after first-line treatment for newly diagnosed PCNSL (CD20+, diffuse large B cell type). Patients were treated with HD-MTX based regimen in conjunction with blood–brain barrier disruption (HD-MTX/BBBD); 44 subsequently relapsed. Images and medical records of these 44 consecutive patients were retrospectively reviewed. The anatomical location of enhancing lesions at initial diagnosis and at the time of relapse were identified and compared.

Results

37/44 patients fulfilled inclusion criteria and had new measureable enhancing lesions at relapse; the pattern and location of relapse of these 37 patients were identified. At relapse, the new enhancement was at a spatially distinct site in 30 of 37 patients. Local relapse was found only in seven patients.

Discussion

Unlike gliomas, the majority of PCNSL had radiographic relapse at spatially distinct anatomical locations within the brain behind a previously intact neurovascular unit (NVU), and in few cases outside, the central nervous system (CNS). This may suggest either (1) reactivation of occult reservoirs behind an intact NVU in the CNS (or ocular) or (2) seeding from bone marrow or other extra CNS sites.

Conclusion

Recognizing patterns of relapse is key for early detection and may provide insight into potential mechanisms of relapse as well as help develop strategies to extend duration of complete response.
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Metadata
Title
Patterns of relapse in primary central nervous system lymphoma: inferences regarding the role of the neuro-vascular unit and monoclonal antibodies in treating occult CNS disease
Authors
Prakash Ambady
Rongwei Fu
Joao Prola Netto
Cymon Kersch
Jenny Firkins
Nancy D. Doolittle
Edward A. Neuwelt
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Fluids and Barriers of the CNS / Issue 1/2017
Electronic ISSN: 2045-8118
DOI
https://doi.org/10.1186/s12987-017-0064-3

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