The clinical heterogeneity of entirely nonenhancing CNS lymphoma: a case series

Bowden, Stephen; Munger, Daniel; Thiessen, Jaclyn; Woll, S. Cody Schoettler; Han, Seunggu J.; Neuwelt, Edward A.; Barajas, Ramon F.; Ambady, Prakash

doi:10.2217/cns-2020-0020

Cited by 4 publications

(4 citation statements)

References 29 publications

(4 reference statements)

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“…As previously mentioned, it is now widely recognized that contrast enhancement is a metric of BBB dysfunction and not a direct representation of tumor burden. In addition, cases of entirely non-enhancing CNS lymphoma have been reported which may delay diagnosis and impeded response assessment ( 17 ).…”

Section: Anatomical Magnetic Resonance Imaging (Mri)mentioning

confidence: 99%

“…PCNSL frequently presents as iso or hypointense (compared to the cortex) lesions on T2W MRI images due to high cellularity that appears distinct from peritumoral edema ( Figure 1 ) ( 19 , 20 ). Clinicians familiar with PCNSL are also aware of progressive white matter changes best seen on T2W MRIs, even when tumors continue to show response based on enhancement ( 17 , 21 ). The risk factors of developing these white matter changes include the elderly (age >60), those receiving neurotoxic chemotherapies, and those who have received prior radiation.…”

Section: Anatomical Magnetic Resonance Imaging (Mri)mentioning

confidence: 99%

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Primary central nervous system lymphoma: advances in MRI and PET imaging

Ambady¹,

Hu²,

Politi³

et al. 2021

Ann Lymphoma

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Contrast enhanced magnetic resonance imaging (CE-MRI) remains the imaging modality of choice for initial workup, staging, and response assessment after therapy in patients with primary central nervous system lymphoma (PCNSL). While CE-MRI is a sensitive test to detect blood brain barrier (BBB) dysfunction, it does not biologically represent the true tumor burden. Current response assessment criteria relies heavily on two dimensional anatomical measurements on post contrast T1-weighted (T1W) images, as well as pre-contrast T2-weighted (T2W) imaging. Additional MRI features, such as diffusion-weighted imaging (DWI) and perfusion weighted imaging, can be routinely obtained at most centers with MRI capabilities. Emerging evidence supports the incorporation of these data to better define tumor physiology and provide additional valuable clinical tools capable of identifying high risk subgroups as well as early predictors of response to therapies. Further, novel advanced molecular and pathophysiologic characterization of PCNSL provides insights into promising targeted therapeutic approaches. However, significant institutional imaging variation and inconsistent clinical trial reporting diminishes the reliability, reproducibility and eventual translation in day to day management of patients with PCNSL. Here we review established neuroimaging concepts and provide an overview of published literature about novel imaging techniques that may improve diagnosis and response assessments. Finally, we highlight the need for standardization of image acquisition, post-processing, and incorporation of novel imaging biomarkers in early phase PCNSL clinical trials.

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Section: Anatomical Magnetic Resonance Imaging (Mri)mentioning

confidence: 99%

Section: Anatomical Magnetic Resonance Imaging (Mri)mentioning

confidence: 99%

Primary central nervous system lymphoma: advances in MRI and PET imaging

Ambady¹,

Hu²,

Politi³

et al. 2021

Ann Lymphoma

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show abstract

“…The former hypothesis is potentially supported by preliminary reports demonstrating subclinical evidence of systemic disease by polymerase chain reaction of the rearranged immunoglobulin heavy-chain genes; in the context of radiological CR ( 16 , 19 , 20 ). The latter hypothesis is better supported based on observations that suggest that relapse may present as a non-enhancing lesion ( 21 – 23 ) and that a majority of relapses occur at spatially distinct anatomical locations within the brain with previously intact BBB ( 16 , 17 ). In this review we outline potential therapeutic approaches and their relative merits, together with the numerous challenges inherent in treating patients with rrPCNSL.…”

Section: Introductionmentioning

confidence: 99%

Relapsed and refractory primary CNS lymphoma: treatment approaches in routine practice

Ambady¹,

Doolittle²,

Fox³

2021

Ann Lymphoma

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Despite recent therapeutic progress and improved survival for many patients with primary central nervous system lymphoma (PCNSL), up to 50% of patients will experience refractory or relapsed disease following first-line treatment with high dose methotrexate (HD-MTX) based regimens. The majority of such events occur within 2 years of diagnosis although, unlike their systemic counterpart, the risk of PCNSL relapse remains, even for patients in radiologic complete response at 10 years following diagnosis. Currently, there are no approved therapies, and no widely accepted ‘standard-of-care’ approaches for the treatment of refractory or recurrent primary central nervous system lymphoma (rrPCNSL). Re-treatment with HD-MTX based regimens, use of non-cross resistant chemotherapy regimens, high-dose chemotherapy and autologous stem cell transplantation (HDT-ASCT), and brain irradiation all remain important therapeutic approaches for rrPCNSL. However, the survival outcomes for patients with rrPCNSL remain extremely poor and the vast majority of patients will die of their disease. Increasingly, novel treatment approaches are being investigated in early phase clinical studies. Importantly, such therapies need to be evaluated in the context of both refractory and relapsed disease; in older patients and those with co-morbid conditions; and those with neurocognitive dysfunction. A deeper understanding of the molecular genetic mechanisms underpinning rrPCNSL and its unique tumor microenvironment is urgently needed to inform biologically rational and effective therapies. rrPCNSL remains a clear unmet clinical need and a high priority area for clinical research that will require national and international collaborative studies with embedded translational science in order to improve outcomes for patients.

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“…Utilizing the characteristics of a low relative cerebral blood volume (rCBV) on perfusion-weighted images and low apparent diffusion coefficient (ADC) values can be helpful in differentiating CNSL from other disease entities [10]. Nonenhancing CNS lymphoma is particularly rare and has been limited to case reports [11]. The delayed diagnosis of CNS lymphoma is often a contributing factor to poor prognoses.…”

mentioning

confidence: 99%

CNS Involvement of DLBCL Presenting with an Unusual Non-Enhancing Infiltrative Mass

Hsueh,

Chen,

Tzeng

2023

Diagnostics

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Central nervous system (CNS) involvement in diffuse large B-cell lymphoma (DLBCL) is relatively uncommon, occurring in approximately 5% of cases, with the majority of instances manifesting during relapse and often associated with poor prognoses. The aim of this case report is to present a unique occurrence of non-enhancing relapse of CNS lymphoma. Significantly, the patient had recently encountered a disease involvement in the axilla region, and subsequent to scheduled chemotherapy, she developed persistent neurological symptoms, leading to the discovery of a relapse of the CNS lymphoma. Our focus will be on delineating the clinical presentation, elucidating the findings observed in clinical imaging, and detailing the therapeutic approaches employed in this specific case. By highlighting these aspects, we aim to provide valuable insights into the diagnosis of the atypical presentation of CNS lymphoma.

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The clinical heterogeneity of entirely nonenhancing CNS lymphoma: a case series

Cited by 4 publications

References 29 publications

Primary central nervous system lymphoma: advances in MRI and PET imaging

Primary central nervous system lymphoma: advances in MRI and PET imaging

Relapsed and refractory primary CNS lymphoma: treatment approaches in routine practice

CNS Involvement of DLBCL Presenting with an Unusual Non-Enhancing Infiltrative Mass

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