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Primary Central Nervous System Lymphoma

Primary Cns Lymphoma


Presentation

  • The aim of the present study was to evaluate morphological MRI characteristics in histologically-proven PCNSL and GBM at the time of their initial presentation.[ncbi.nlm.nih.gov]
  • Primary CNS lymphoma can present as an acute massive ICH, and immunohistochemistry in the present study strengthened the suggestion that VEGF activity is related to the hemorrhage in primary CNS lymphoma.[ncbi.nlm.nih.gov]
  • Hemorrhage at presentation in primary central nervous system (CNS) lymphoma is rare. We encountered a case of primary CNS lymphoma presenting as a growing intracerebral hemorrhage. An 80-year-old man presented with mild dysarthria.[ncbi.nlm.nih.gov]
  • 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]
  • Three patients presented a pattern of marked decrease of NAA/Cr, increase of Cho/Cr, and two of the three cases showed increased Lip/Cr and Lac/Cr on MRS.[ncbi.nlm.nih.gov]
Hodgkin Lymphoma
  • Herein, for the first time, we describe the radiologic and pathologic features of a primary CNS posttransplantation lymphoproliferative disease that reveals Epstein-Barr virus-positive Hodgkin lymphoma.[ncbi.nlm.nih.gov]
  • Primary central nervous system lymphoma (PCNSL) is an uncommon form of non-Hodgkin lymphoma affecting the brain, spinal cord, and leptomeninges.[ncbi.nlm.nih.gov]
  • Gonzalez GD, Schuster-Uitterhoeve ALJ: Primary non-Hodgkin's lymphoma of the central nervous system. Cancer 51: 2048–2052, 1983 Google Scholar 12.[doi.org]
  • Although the rate of increase in the incidence of non-Hodgkin's lymphoma of the brain is higher than for peripheral non-Hodgkin's lymphoma and other glial tumors, brain lymphoma is not projected to surpass glial tumors as the most frequently diagnosed[ncbi.nlm.nih.gov]
  • Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil.[ncbi.nlm.nih.gov]
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]
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]
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]
  • We report a case of primary central nervous system lymphoma presenting as multiple intraventricular masses in an immunocompetent 68 year old man with severe headache and unsteady gait.[ncbi.nlm.nih.gov]
  • A previously healthy 54-year-old woman presented with weight loss, progressive weakness that was more pronounced on the left side, and intermittent occipital headaches.[ncbi.nlm.nih.gov]
  • The patient had a headache two months after admission and the magnetic resonance image of the brain showed left frontal lobe hypodensity.[ncbi.nlm.nih.gov]
  • A 45-year-old woman presented with headache, and then rapidly developed a third nerve palsy and bitemporal hemianopsia.[ncbi.nlm.nih.gov]
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]
  • A 16-year-old female patient presented to the clinic with complaints of multiple episodes of generalized tonic clonic seizures, nystagmus, and weakness on the left side of the body for 3 weeks.[ncbi.nlm.nih.gov]
  • Anticonvulsants may be given if seizures occur as a result of a tumour.[leukaemia.org.au]
  • 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]
  • This pressure can lead to headaches, vomiting, vision and balance problems, as well as seizures. Lesions that originate in the spinal cord can lead to difficulty walking, urinating, or moving your bowels.[cumc.columbia.edu]
Confusion
  • A 58-year-old woman was admitted with confusion and left hemiparesis.[ncbi.nlm.nih.gov]
  • 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]
  • Depend on where the tumor is, the symptoms can include: Throwing up or feeling like you’re going to Weakness in your legs and arms Seizures Headaches Confusion Double vision Hearing loss Trouble swallowing Trouble with balance and coordination Continued[webmd.com]
Focal Neurologic 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]
  • The most common presenting symptom is progressive focal neurologic deficit, which is seen in 70% of patients.[clinicaladvisor.com]
  • In our case, the patient presented with focal neurological deficit with a history of recent dental procedure, use of long-term immunosuppressive therapy, and contrast rim-enhancing lesion on CT.[hkmj.org]
  • 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]
Stupor
  • 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]
  • Progressive dementia or stupor with no focal signs and with little or no enhancement on MRI may be more common in patients with AIDS who have PCNSL.[emedicine.medscape.com]

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]
  • The systemic lymphoma workup was essentially normal. She received 5 cycles of chemoimmunotherapy with rituximab, high-dose methotrexate (HDMTX), vincristine, and procarbazine and had a complete radiological response (CR).[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]
  • Despite high tumor response rates to initial treatment, many patients will relapse. The choice of salvage treatment will depend on age, previous treatment and response, performance status, and comorbidities at the time of relapse.[ncbi.nlm.nih.gov]
  • Nivolumab could be a novel treatment for intractable recurrent PCNSL in the future.[ncbi.nlm.nih.gov]
  • Currently, the initial treatment of choice incorporates high-dose methotrexate (HD-MTX) either as a single agent or in combination with other systemic chemotherapies.[ncbi.nlm.nih.gov]
  • In survival analysis, for patients with DLBCL, chemotherapy involving treatments was associated with the most favorable survival. Received-only radiation could be considered as a primary treatment in immunocompetent patients with non-DLBCL.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis remains poor despite the inclusion of treatment with rituximab during the study period.[ncbi.nlm.nih.gov]
  • 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]
  • 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.[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]
  • The important biomarkers indicating prognosis are not identified. Multicenter studies should be carried out to elucidate pathogenesis of PCNSL to find novel and effective therapeutic strategies.[ncbi.nlm.nih.gov]

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]
  • PURPOSE: Primary central nervous system lymphoma (PCNSL) is a type of non-Hodgkin lymphoma (NHL), and it has been postulated that metabolic disorder may contribute to NHL etiology.[ncbi.nlm.nih.gov]
  • 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]
  • OBJECTIVES: This study aims to characterize the epidemiology of immunocompetent Primary central nervous system lymphoma (PCNSL) diagnosed 2000-2013 in Sweden. METHODS: Cases were identified in the population-based Swedish Lymphoma Register.[ncbi.nlm.nih.gov]
  • In Part 1 of our two-part coverage of this entity, we provided an overview of the epidemiology of primary CNS lymphoma, followed by a discussion of the diagnostic and staging evaluation, and a review of current prognostication systems.[ncbi.nlm.nih.gov]
  • The current analysis drew from the updated Surveillance, Epidemiology, and End Results (SEER) database to establish projections for the incidence of brain lymphoma.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed.[ncbi.nlm.nih.gov]
  • Novel insights into the pathophysiology of PCNSL have identified the B-cell receptor (BCR) pathway as a key mechanism in the pathogenesis of PCNSL.[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]
  • How can Primary Central Nervous System Lymphoma be Prevented? Currently, it is not possible to prevent Primary Central Nervous System Lymphoma. However, controlling certain factors my help lower one’s risk for the condition.[dovemed.com]
  • Our physicians value the relationship they have with each of their patients and encourage each patient to play an active role in disease prevention and management.[sjo.org]

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