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Primary Central Nervous System Lymphoma
Primary Cns Lymphoma

Images

WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0

Presentation

Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal manifestation of non-Hodgkin's lymphoma with those presenting at the cerebellopontine angle (CPA) being rare presentations with limited reported cases in the literature. [ncbi.nlm.nih.gov]

Entire Body System

  • Hodgkin Lymphoma

    The lesion showed characteristic Hodgkin-Reed Sternberg cells with a typical immunophenotype, accompanied by numerous nonneoplastic B cells and T cells demonstrating classic Hodgkin lymphoma within the brain. [ncbi.nlm.nih.gov]

    Epidural metastases occur in 0.1%-6.5% of patients with non-Hodgkin’s lymphoma. 8 9 Although epidural non-Hodgkin’s lymphoma does not directly involve the CNS, patients present with back pain and symptoms and signs of myelopathy. [jnnp.bmj.com]

    PD-1 blockade has shown safety and encouraging results in the treatment of Hodgkin’s lymphoma patient and is promising in the treatment of non-Hodgkin’s lymphoma. [neurology.org]

  • Fatigue

    Less common side effects may include convulsion, severe headache, severe fatigue, low blood count, infection. Rare side effects may include pneumonia due to Pneumocystis carinii organism. Cytarabine (Ara-C) is an intravenous chemotherapy drug. [sjo.org]

    The cause of the condition is unknown, though genetic mutations have been implicated The condition can cause headaches, fatigue, convulsions, and other general signs and symptoms, such as fever, weight loss, and appetite loss. [dovemed.com]

    Some experience fatigue, headache and inflammation of the external auditory canal or middle ear • Patients requiring treatment of the eye are likely to experience conjunctival irritation and dry eye. • These acute effects typically resolve within 6 – [slideshare.net]

    The side effects of whole-brain radiotherapy can include: worsening of your symptoms in the early stages of treatment fatigue – this might take some months to improve after treatment poor attention poor memory problems with language slowness of thinking [lymphomas.org.uk]

Eyes

  • Diplopia

    We report a 66-year-old man with 2-month history of dizziness and 1-month history of diplopia. Cranial magnetic resonance imaging (MRI) disclosed two solid masses, one in the right lateral ventricle and another in the fourth ventricle. [ncbi.nlm.nih.gov]

    Systemic symptoms may include fever, night sweats, or weight loss.Other symptoms include diplopia dysphagia vertigo monocular vision loss progressive dementia or stupor in patients with a nonfocal neurologic exam and minimal abnormalities on MRI (more [en.wikipedia.org]

    N/A A homogeneously enhancing mass within the fourth ventricle without hydrocephalus Excision Diffuse large B-cell lymphoma Chemotherapy and WBRT 6 months without recurrence Gossmanet al. [8]2014 66/M Progressive gait disturbances and diplopia N/A A [omicsonline.org]

    Such patients give a history of (1) blurred vision, (2) headache, (3) isolated cranial nerve dysfunction (eg, diplopia, dizziness, dysphagia, facial numbness, monocular visual loss), or (4) spinal nerve root symptoms (eg, pain, dysfunction localized to [emedicine.medscape.com]

Neurologic

  • Headache

    No headache was reported on admission, but previously she had intermittent mild headaches and generalized body aches. She had no history of visual symptoms. [ncbi.nlm.nih.gov]

    Case Report A 61-year-old man presented to our hospital complaining of headache for one month and somnolence, abnormal gait and urinary incontinence for one week. [omicsonline.org]

  • Seizure

    The boy had a history of walking weakness and seizure for 4 months. The serum levels of immunoglobulin G, A and M were decreased. Brain MRI showed multiple lesions which had ring enhancement. CT showed calcification in all of the lesions. [ncbi.nlm.nih.gov]

    Symptoms Non-focal, non-specific symptoms occur in more than 50 percent of patients; mental status changes in one third; symptoms of increased intracranial pressure (headache, nausea/vomiting) and/or generalized seizures in 9 percent. [neurosurgery.ucla.edu]

    He was treated with highly antiretroviral therapy (HAART) alone and his general condition improved and the seizures diminished. Key words. [vzmechani.blogspot.com]

    Anticonvulsants may be given if seizures occur as a result of a tumour. [leukaemia.org.au]

  • Confusion

    Her family said she was mildly confused and had memory difficulties that started acutely 2 days before presentation to the hospital. [ncbi.nlm.nih.gov]

    Symptoms and signs of PCNSL vary, depending on the area of the brain that is involved and include severe headaches, changes in speech, personality changes, confusion, memory problems, drowsiness, muscle weakness, and numbness in the extremities. [secure.ssa.gov]

    Common symptoms of PCNSL are: Headaches Seizures Changes to or difficulty with speech Drowsiness Memory loss and frequent confusion Loss of feelings in the extremities Changes in habits or demeanor Changes in vision including spots and vision loss These [disability-benefits-help.org]

  • Focal Neurological Deficit

    A primary CNS lymphoma usually presents with seizure, headache, cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect. [en.wikipedia.org]

    Conclusions : PCNSL presents most commonly in the sixth decade with focal neurological deficit, behavioral symptoms and cognitive decline. High grade DLBCL is the commonest histological subtype. [neurologyindia.com]

    The most common presenting symptom is progressive focal neurologic deficit, which is seen in 70% of patients. [clinicaladvisor.com]

    Patients with PCNSL present with focal neurological deficits, neuropsychiatric symptoms, signs of raised intracranial pressure, and seizures. [ci.nii.ac.jp]

    Patients with this diagnosis often present similarly to those diagnosed with other brain malignancies, and include focal neurologic deficits, neurocognitive symptoms, headaches, seizures, and ocular symptoms. [acanceresearch.com]

  • Cognitive Impairment

    The serial Montreal Cognitive Assessment evaluations (20-72 months post chemotherapy) of 10 long-term CR patients revealed one dementia among three patients without WBRT and five mild cognitive impairments in seven patients with WBRT. [ncbi.nlm.nih.gov]

    Hemodynamics Miscellaneous Mitral Regurgitation Mitral Stenosis PCI and Cardiac Surgery Pre-operative Assessment Risk Scores Shunts Syncope Treadmill Testing Body Imaging Obstetrical imaging Thrombosis imaging Trauma/MSK imaging REFERENCE BOOK Screening for Cognitive [qxmd.com]

    While it often results in better disease control it comes with significant risk of cognitive problems. Over time studies have shown that while it may initially lead to superior progression free survival the cognitive impairment is too substantial. [nhlcyberfamily.org]

    Treatment induced neurotoxicities include vincristine neuropathy, ifosfamide or cytarabine encephalopathy, radiation myelopathy, or radiation related cognitive impairment. [jnnp.bmj.com]

    Neurotoxicity can occur and is especially notable for patients older than 60 years, who can develop cognitive impairment after standard doses of whole-brain radiation. [hematologyandoncology.net]

Workup

Postsurgical pathologic workup revealed malignant B cell lymphoma with morphologic features of diffuse large B cell lymphoma. Eight of these 11 patients with PCNSL showed a clear fluorescence induced by 5-ALA. [ncbi.nlm.nih.gov]

Consultations Ophthalmologic consultation is required as part of the initial workup and staging of PCNSL, in order to exclude vitreous involvement. [emedicine.medscape.com]

Treatment

The optimal treatment for refractory or relapsed PCNSL is poorly defined. The choice of salvage treatment depends on a patient's age, previous treatment and response, performance status, and comorbidities at the time of relapse. [ncbi.nlm.nih.gov]

He started treatment for refractory disease with rituximab and nivolumab to which he had a complete response radiographically after 2 cycles of treatment. [neurology.org]

Prognosis

The overall prognosis, diagnosis, and management of PCNSL differ from those for other types of NHL. Prompt diagnosis and initiation of treatment are vital for improving clinical outcomes. [ncbi.nlm.nih.gov]

Objective: To compare the pathogenesis, clinical presentation, therapy, and prognosis of primary central nervous system lymphoma in immunocompetent persons with these characteristics of the disease in patients with AIDS. [doi.org]

Etiology

Primary central nervous system lymphoma (PCNSL) is a rare malignancy that usually arises in the context of severe immunosuppression but has incompletely understood etiology, limiting effective treatments. [ncbi.nlm.nih.gov]

Epstein-Barr virus as a possible etiologic agent in primary central nervous system lymphoma in immunocompetent individuals. Neurol India 2009;57:36-40. [ PUBMED ] 29. Glass J, Gruber ML, Cher L, Hochberg FH. [neurologyindia.com]

Etiology of CNS lymphomas As for most other types of NHL, the etiology of CNS lymphomagenesis is largely undefined and the mechanistic basis for brain tropism is not understood. [bloodjournal.org]

Etiology The DLBCL type of PCNSL is composed of immunoblasts or centroblasts that have a predilection for blood vessels. Lymphoid clustering around small cerebral vessels is typically seen. [emedicine.medscape.com]

Epidemiology

The epidemiology, therapeutic measures, and clinical characteristics were listed as descriptive statistics. [ncbi.nlm.nih.gov]

This introduction is followed by chapters on the pathology, epidemiology, diagnosis and management of PCNSL. These chapters review PCNSL in “immunocompetent” hosts as well as in the setting of immunosuppression. [books.google.com]

Pathophysiology

Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed. [ncbi.nlm.nih.gov]

There are several hypotheses to explain the origin of B cell primary CNS lymphoma but no definitive data which explain their pathophysiology. [jnnp.bmj.com]

Pathophysiology At least 95% of PCNSL are B-cell neoplasms which express the B-cell marker CD20. [oncologynurseadvisor.com]

Histopathology and molecular pathophysiology PCNSLs are mature B-cell lymphomas characterized by the expression of pan B-cell markers (CD20 and CD79a) (Figure 2). Typically, they have a high proliferation rate (Ki-67 proliferative index >70%). [aerzteblatt.de]

Prevention

From prevention and early detection strategies to treatment, supportive care and survivorship, the Cancer Institute is here for you. [utmedicalcenter.org]

You may have anti-convulsant drugs to prevent seizures (fits). The main treatment for PCNSL is usually chemotherapy. It is often given with a targeted therapy drug called rituximab. [macmillan.org.uk]

Prevention of neurological complications with better systemic therapy and, possibly, prophylactic intrathecal chemotherapy are areas of active investigation. [books.google.com]

Seite 290 - Antibiotic prophylaxis for the prevention of bacterial infections in cirrhotic patients with gastrointestinal bleeding: A meta-analysis. Hepatology 1999; 29: 1655-61 6. ‎ [books.google.de]

REFERENCE BOOK Screening for Colorectal Cancer | CTFPHC REFERENCE BOOK Screening for Cognitive Impairment | CTFPHC REFERENCE BOOK Screening for Depression | CTFPHC REFERENCE BOOK Screening for Developmental Delay | CTFPHC Review Canadian Task Force on Preventive [qxmd.com]

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