Primary CNS Lymphomas: Challenges in Diagnosis and Monitoring

Chiavazza, Carlotta; Pellerino, Alessia; Ferrio, F; Cistaro, Angelina; Soffietti, Riccardo; Rudà, Roberta

doi:10.1155/2018/3606970

Cited by 96 publications

(134 citation statements)

References 133 publications

(148 reference statements)

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“…Primary Central Nervous System Lymphoma is a rare extranodal NHL, involving the brain, eye, leptomeningitis, and rarely spinal cord. [ 3 , 14 ] The diagnosis of CNS lymphoma is always challenging. Presently, it is mainly based on clinical characteristics, imaging, CSF analysis, and brain biopsy.…”

Section: Discussionmentioning

confidence: 99%

Cell-Block cytology in diagnosis of primary central nervous system lymphoma

Huang

Li-ling²,

Tong³

2020

Medicine

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Introduction: Primary Central Nervous System Lymphoma (PCNSL) remains a diagnostic challenge due to the variable clinical manifestations. Liquid biopsies, particularly those involving cell-free DNA (cfDNA) from plasma, are rapidly emerging as important and minimally invasive adjuncts to traditional biopsies. However, conventional pathology may be still essential to obtain a diagnosis. Patient concerns: A 56-year-old woman presented with a progressive headache, dizziness, blurred vision, and lower limbs weakness with dysesthesia. Atypical clinical and radiological presentations, previous empirical treatment in another hospital, together with the patient's refusal to stereotactic brain biopsy made it challenging to diagnose. Her status deteriorated continuously during hospitalization. Diagnosis: Lumber punctual was performed, and CSF cytological analysis revealed malignancy cells with a high nuclear-cytoplasmic ratio. However, these cells were too loose to perform immunohistochemical stains. Genetic aberrations detections with CSF and peripheral blood sample were also inconclusive. We made a “cell-block” using the sedimentary cells collected from CSF collected through multiple aspirations via an Omaya reservoir. We further performed cytopathological and immunohistochemical analysis using this “cell-block,” which finally confirmed the diagnosis of diffuse large-B cell PCNSL. Interventions: Intracranial chemotherapy began afterwards (MTX 15 mg and dexamethasone 5 mg, twice per weeks). Outcomes: Unfortunately, this patient was dead 2 weeks later due to severe myelosuppression and secondary septic shock. Conclusion: We provided “cell-block” method, which collects cell components from large amount of CSF for cytology and immunohistochemical analysis. “Cell-block” cytology can be an alternative diagnostic method in diagnosis of PCNSL.

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Section: Discussionmentioning

confidence: 99%

Cell-Block cytology in diagnosis of primary central nervous system lymphoma

Huang

Li-ling²,

Tong³

2020

Medicine

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“…Flanagan et al reported that spinal cord 18 F-FDG-PET/CT hypermetabolism in patients with active myelopathy was much more common than in ammatory myelopathies (12). PCNSL has a strong uptake of 18 F-FDG, while the 18 F-FDG uptake is not obvious in non-enhancing lesions on MRI (13).…”

Section: Discussionmentioning

confidence: 99%

An overlooked cause of longitudinally extensive spinal cord lesions: Primary central nervous system lymphoma

Wang¹,

Qi²,

Han³

et al. 2020

Preprint

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Background: Primary central nervous system lymphoma (PCNSL ) is a rare and aggressive malignant tumor. It is easy to be misdiagnosed due to its low incidence and unspecific presentations in clinical practice. PCNSL mainly occurs intracranially in the brain while spinal cord is rarely involved. Case presentation: Here we report a 76-year-old woman who had a suspicious tumor history and presented retardant paralysis, bladder dysfunction and sensory loss of the lower limbs. Magnetic resonance imaging (MRI) of the thoracic spine disclosed longitudinally extensive lesions extending from thoracic 4 (T4) to lumbar 1 (L1) vertebral level with an enhanced nodular lesion noting at levels of T10 and T11 . In order to further identify the cause, the whole body 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET)/computed tomography (CT) was performed and showed a hypermetabolic nodule corresponded to MRI enhancing lesions, which further suggesting the possibility of a tumor. The patient then underwent a surgical resection and spinal cord biopsy confirmed the diagnosis of non-Hodgkin's lymphoma (diffuse large B-cell type). The patient then received a high-dose chemotherapy based on methotrexate combined with Rituximab. Unfortunately, the symptoms of this patient have not been improved significantly after three rounds of chemotherapy. Conclusion: Our case indicates that PCNSL may also serve as a possible cause for longitudinally extensive spinal cord lesions, especially the patients who had a suspicious tumor history, MRI enhancing lesion s in the spinal cord corresponded to hypermetabolic nodules on 18 F-FDG- PET/CT at the same level.

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“…Whole-body 18 F-FDG-PET/CT can be performed to detect sys- temic lymphoma before corticosteroid administration in a PCNSL-suspected patient who has difficulty undergoing brain biopsy, as systemic involvement of DLBCL is found in 7% of PCNSL-suspected patients at initial diagnosis [11]. However, studies on the usefulness of 18 F-FDG-PET/CT in differentiation of PCNSL from IDD is lacking [12]. The key radiologic features that help distinguish PCNSL with spinal cord involvement from IDD are: (1) multifocal intramedullary lesions with brain lesions [5]; (2) involvement of the conus medullaris or cauda equine [5]; (3) persistent Gd-enhancing intramedullary lesions for over 8 weeks [5]; (4) persistent, contiguous Gd-enhancing MRI lesions along the ventricular regions and spinal leptomeninges [4]; and (5) intraparenchymal tadpole-like enhancing lesions, which may be helpful in suspected PCNSL cases.…”

Section: Discussionmentioning

confidence: 99%

Primary central nervous system lymphoma with intramedullary spinal cord involvement mimicking inflammatory demyelinating disease

Kim¹,

Nam²,

Lévy³

et al. 2019

J Neurocrit Care

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Background: Spinal cord involvement of primary central nervous system lymphoma (PCNSL) is rare in a young immunocompetent patient and can be misdiagnosed as an inflammatory demyelinating disease (IDD) of the central nervous system. Case Report: We report a case of PCNSL mimicking IDD in a previously healthy 46-year-old man with weakness in both hands for 1 week. Magnetic resonance imaging (MRI) of the cervical spinal cord revealed contrast-enhancing intraparenchymal and leptomeningeal lesions in the cervical spinal cord and medulla oblongata. Cerebrospinal fluid analysis revealed pleocytosis (37/mm 3). The patient's symptoms and lesions improved with corticosteroid treatment. However, he developed semicomatose mentality 5 months later. Brain MRI, ventricular biopsy, and 18 F-flurodeoxyglucose positron emission tomography/computed tomography confirmed PCNSL. The patient deceased 3 months later, despite high-dose methotrexate chemotherapy. Conclusion: Persistent gadolinium-enhancing MRI lesions along the ventricular regions and spinal leptomeninges could differentiate PCNSL involving the spinal cord from IDD in the early stages of the disease.

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Primary CNS Lymphomas: Challenges in Diagnosis and Monitoring

Cited by 96 publications

References 133 publications

Cell-Block cytology in diagnosis of primary central nervous system lymphoma

Cell-Block cytology in diagnosis of primary central nervous system lymphoma

An overlooked cause of longitudinally extensive spinal cord lesions: Primary central nervous system lymphoma

Primary central nervous system lymphoma with intramedullary spinal cord involvement mimicking inflammatory demyelinating disease

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