Different facets of intracranial central nervous system lymphoma and its imaging mimics

Kwok, Hoi Ming; Li, K.Y.; Chan, Rois L S; Chan, Chung-hong; Wong, Stephen Ka Hon; Lee, Chiu Man; Cheng, Lik Fai; Fai, Johnny Ka

doi:10.25259/jcis_135_2021

Cited by 11 publications

(8 citation statements)

References 26 publications

(117 reference statements)

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“…[ 9 ] In immunocompromised patients, lesions with irregular margins, heterogeneity, annular enhancement, and heterogeneous peripheral areas with a center of low attenuation due to necrosis tend to be more multifocal and are also surrounded by a greater degree of vasogenic edema occur. [ 14 , 17 ] In diffusion studies, it presents an evident diffusion restriction and has lower ADC values than gliomas. The MRI spectral pattern in PCNSL shows a decrease in the NAA/Cho and NAA/Cr ratios and an increase in the Cho/Cr ratios, Cho/NAA, as well as the presence of lactate and lipid peaks.…”

Section: Discussionmentioning

confidence: 99%

Primary central nervous system lymphoma: A mirror type presentation in an immunocompetent patient

Chávez,

Arce,

Perea

et al. 2024

Surgical Neurology International

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Background: Primary central nervous system (CNS) lymphoma is a very rare extranodal non-Hodgkin lymphoma. The bilateral pattern, as we call it “mirror type”, has been identified in other CNS lesions such as gliomas, metastases, and demyelinating lesions, so the differential diagnosis includes imaging studies such as magnetic resonance imaging contrasted with spectroscopy, ruling out immunodeficiency or metastatic disease. Case Description: A 65-year-old female presented progressing headache, loss of memory and language alterations, as well as sensory alterations. Neuroimaging showed the presence of two equidistant periventricular lesions at the level of both ventricular atria, a spectroscopy study suggestive of malignancy. Serological studies showed no evidence of immunodeficiency or the presence of positive tumor markers; however, a biopsy was performed, which revealed a histopathological result of primary lymphoma of the CNS. Conclusion: In neuro-oncology, primary CNS tumors with multiple lesions are rare, even more, the “mirror type” lesions. Lymphomas are lesions that can present in different ways on imaging and clinical presentation. These tumors that present a vector effect due to their size, perilesional edema, or that lead to loss of neurological function are highly discussed in diagnostic and surgical treatment. Due to their prognosis, action on diagnosis and treatment must be taken as quickly as hospital resources allow.

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Section: Discussionmentioning

confidence: 99%

Primary central nervous system lymphoma: A mirror type presentation in an immunocompetent patient

Chávez,

Arce,

Perea

et al. 2024

Surgical Neurology International

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“…The disease in immunocompetent patients typically presents as a solitary lesion with predilection for the periventricular white matter, with possible involvement of the gray matter [ 6 ]. Brain MRI plays an important role in the diagnosis of this entity, which is characterized by restricted diffusion and T2 hypointense signal, suggesting hypercellularity [ 7 ]. On per-fusion study, these lesions show lower rCBV compared with high-grade glioma, supporting this differential diagnosis [ 7 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

Rodrigues,

Santos,

Martins

2024

Am J Case Rep

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Patient: Male, 44-year-old Final Diagnosis: Primary central nervous system diffuse large B-cell lymphoma Symptoms: Bilateral frontal headache Clinical Procedure: — Specialty: General and Internal Medicine • Neurosurgery • Radiology Objective: Unusual clinical course Background: Primary central nervous system diffuse large B-cell lymphoma (DLBCL) is an extremely aggressive brain disease that rarely affects immunocompetent non-elderly patients, particularly with hemorrhagic presentation. Brain magnetic resonance imaging (MRI) plays an important role in the diagnosis of this entity, which typically demonstrates restricted diffusion and a T2 hypointense appearance, suggesting hypercellularity. Case Report: A 44-year-old man came to the emergency department with a persistent and treatment-resistant bilateral frontal headache that had been bothering him for the past 3 weeks. Upon conducting a neurological assessment, the patient displayed temporal disorientation and incoherent speech, but without any observable motor deficits. A non-contrast enhanced brain computed tomography scan was carried out, revealing a hyperattenuating, space-occupying lesion and hemorrhage in the left hemisphere of the brain. Subsequently, brain MRI demonstrated hypointense signal on T2-weighted images, restricted diffusion, and homogeneous lesional contrast enhancement, suggesting a very cellular expansive lesion with hemorrhage. To establish a definitive diagnosis, a brain biopsy was undertaken, confirming the presence of DLBCL of the primary central nervous system (germinal center phenotype). Conclusions: Hemorrhagic presentation of primary central nervous system DLBCL occurs very rarely, particularly in non-elderly immunocompetent patients. Brain MRI plays an important role in the diagnosis of this entity, which allows differentiation from high-grade glial or other lesions that present more frequently with hemorrhage. Therefore, it is crucial to suspect lymphoma before surgical intervention for appropriate patient management.

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“…19 Isolated leptomeningeal disease often has a wide differential diagnosis which can include infections such as toxoplasmosis and tuberculosis, autoimmune conditions such as neurosarcoidosis and systemic lupus erythematosus (SLE). 20 21…”

Section: Diagnosismentioning

confidence: 99%

Central Nervous System Lymphoma

Shah,

Venur

2023

Semin Neurol

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Central nervous system lymphoma (CNSL) is a rare and aggressive malignancy that primarily affects the brain, spinal cord, and meninges. This article provides a comprehensive overview of the current understanding of CNSL encompassing its epidemiology, pathophysiology, clinical presentation, diagnosis, treatment modalities, and prognosis. Although the main focus is on primary CNS lymphoma (PCNSL), ocular lymphoma, primary leptomeningeal lymphoma, and secondary CNS lymphoma are also discussed. The pathobiology of CNSL involves the infiltration of malignant lymphocytes within the CNS parenchyma or leptomeninges. Various risk factors and immunological mechanisms contribute to its development, including immunodeficiency states, chronic inflammation, and genomic alterations. Accurate diagnosis is crucial for appropriate management, given the heterogeneous clinical presentation. The neuroimaging, systemic imaging, and other modalities for diagnosis and evaluation for extent of disease involvement will be discussed. Additionally, the importance of histopathological examination, cerebrospinal fluid (CSF) analysis, and molecular testing in confirming the diagnosis and guiding treatment decisions are highlighted. The treatment landscape for CNSL has evolved significantly. Therapeutic approaches encompass a multimodal strategy combining high-dose methotrexate-based chemotherapy, consolidation with whole-brain radiation therapy, and high-dose chemotherapy with stem cell rescue. Recent advancements in targeted therapies and immunomodulatory agents offer promising avenues for future treatment options. We review the clinical outcomes and prognostic factors influencing the survival of CNSL patients, including age, performance status, disease stage, and genetic abnormalities.

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Different facets of intracranial central nervous system lymphoma and its imaging mimics

Cited by 11 publications

References 26 publications

Primary central nervous system lymphoma: A mirror type presentation in an immunocompetent patient

Primary central nervous system lymphoma: A mirror type presentation in an immunocompetent patient

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

Central Nervous System Lymphoma

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