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Primary central nervous system lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Synonyms and keywords: Primary CNS lymphoma; Primary central nervous system lymphomas; Primary CNS lymphomas; Primary lymphoma of the brain; Primary lymphoma of brain; PCNSL; PCNSLs; Microglioma; Diffuse histiocytic lymphoma; Lymphomatosis cerebri; Primary brain lymphoma; Primary diffuse large B-cell lymphoma of the central nervous system; Primary DLBCL of the central nervous system; Primary DLBCL of the CNS; DLBCL-CNS; Primary intraocular lymphoma; Lymphoid malignancy; Extranodal lymphoma

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

Primary CNS lymphoma is an intracranial tumor usually present in those with severe immunosuppression. It represents around 20% of all cases of lymphomas in HIV infection (other types being Burkitt’s lymphoma and immunoblastic lymphoma). Primary central nervous system lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps. EBV is almost always associated with primary CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it’s immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination. . Primary central nervous system lymphoma constitutes approximately 3%-4% of all primary brain tumors and 1%-2% of all lymphomas. Primary central nervous system lymphoma has been diagnosed in at least 2% of individuals infected with human immunodeficiency virus, and in 9–14% of acquired immunodeficiency syndrome (AIDS)-autopsies. Primary CNS lymphoma is, after toxoplasmosis, the most common cause of focal brain lesions in AIDS patients. The overall incidence of primary central nervous system lymphoma is 0.4 per 100,000 individuals per year. The median age of occurrence of primary central nervous system lymphoma in immunocompetent and immunocompromised patients are 53-57 years and 31-35 years, respectively. The peak incidence is between 60 and 70 years old for immunocompetent patients. Significant increment of incidence rate over time is associated with increased incidence of AIDS and advanced age. Males are more commonly affected with primary central nervous system lymphoma than females.

Classification

Primary central nervous system lymphoma may be classified according to the site of involvement of the central nervous system into 3 groups:

  1. Parenchymal
  2. Intravascular lymphoma
  3. Primary leptomeningeal lymphoma.

Primary CNS lymphomas are extranodal, malignant non-Hodgkin lymphomas of the diffuse large B-cell type that are confined to the brain, eyes, leptomeninges, or spinal cord, in the absence of systemic lymphoma. Primary CNS lymphomas are estimated to account for up to 1% of all lymphomas, 4–6% of all extranodal lymphomas, and about 3% of all CNS tumours. After a continuous rise in the incidence of primary CNS lymphoma during the 1980s and 1990s, epidemiological data in high-income countries show a decrease in incidence, particularly among young patients with AIDS.

Pathophysiology

  • Primary central nervous system lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps.
  • EBV is almost always associated with primary CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it’s immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination.
  • Genes involved in the pathogenesis of primary central nervous system lymphoma include IG, BCL6, CD95, PAX5, and PIM1.
  • Primary central nervous system lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and iatrogenically immunosuppressed).
  • Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions). On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage.
  • On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels. The atypical cells have large round, oval and angulated hyperchromatic and vesicular nuclei, and a narrow rim of pale cytoplasm. An occasional mitotic figure is noted.
  • Primary central nervous system lymphoma is demonstrated by positivity to tumor markers, including B lymphocyte markers, such as CD19, CD20, CD79a, Ki-67, GFAP, BCL-2, and BCL-6.
  • Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions).[5][2] On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage.[2] On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels.

Causes

There are no known direct causes for primary central nervous system lymphoma. To view a comprehensive list of risk factors that increase the risk of primary nervous system lymphoma, click here.

Differentiating Primary Central Nervous System Lymphoma from other Diseases

Primary central nervous system lymphoma must be differentiated from secondary CNS lymphoma, cerebral toxoplasmosis, glioblastoma multiforme, cerebral abscess, and tuberculoma.

Epidemiology and Demographics

Primary central nervous system lymphoma constitutes approximately 3%-4% of all primary brain tumors and 1%-2% of all lymphomas. Primary central nervous system lymphoma has been diagnosed in at least 2% of individuals infected with human immunodeficiency virus, and in 9–14% of acquired immunodeficiency syndrome (AIDS)-autopsies. Primary CNS lymphoma is, after toxoplasmosis, the most common cause of focal brain lesions in AIDS patients. The overall incidence of primary central nervous system lymphoma is 0.4 per 100,000 individuals per year. The median age of occurrence of primary central nervous system lymphoma in immunocompetent and immunocompromised patients are 53-57 years and 31-35 years, respectively. The peak incidence is between 60 and 70 years old for immunocompetent patients. Significant increment of incidence rate over time is associated with increased incidence of AIDS and advanced age. Males are more commonly affected with primary central nervous system lymphoma than females. The male to female ratio is approximately 1.2 to 1 in immunocompetent patients and approximately 7.38 to 1 in AIDS-associated primary central nervous system lymphoma.

Risk Factors

The most potent risk factor in the development of primary central nervous system lymphoma is a weakened or suppressed immune system in individuals who have acquired immunodeficiency syndrome (AIDS), received an organ transplant and are on immunosuppressants, inherited immunosuppressive disorders (IgA deficiency, Wiskott-Aldrich syndrome, ataxia telangiectasia), and autoimmune disorders (systemic lupus erythematosus, rheumatoid arthritis, myasthenia gravis).

Screening

There is insufficient evidence to recommend routine screening for primary central nervous system lymphoma.

Natural History, Complications and Prognosis

If left untreated, primary central nervous system lymphoma may progress to develop elevated intracranial pressure, ocular symptoms, focal neurological deficits, and neuropsychiatric problems. Common complications of primary central nervous system lymphoma include relapse, extracranial or subcutaneous metastasis, neuropsychiatric problems, and neurological toxicity. The prognosis of primary central nervous system lymphoma is generally poor.

Diagnosis

Staging

There is no established system for the staging of primary central nervous system lymphoma.

Symptoms

Symptoms of primary central nervous system lymphoma are identical to the other types of brain tumors and depend on the area of the brain that is affected. Primary central nervous system lymphoma is multifocal. Symptoms of primary nervous system lymphoma include headache, nausea, vomiting, difficulty swallowing, monocular vision loss, Muscle weakness or paralysis, memory loss, facial hypoesthesia, seizures, fever, night sweats, and weight loss.

Physical Examination

Common physical examination findings of primary central nervous system lymphoma include vision loss, papilledema, altered mental status, apathy, depression, aphasia, cranioneuropathies, ataxia, and hemiparesis.

Laboratory Findings

Laboratory tests performed in a case suspected of primary central nervous system lymphoma include complete blood count, complete metabolic panel, lactate dehydrogenase, serological testing for HIV, and CSF analysis. Laboratory findings consistent with the diagnosis of primary central nervous system lymphoma include elevated protein and decreased glucose on CSF analysis. Positive EBV DNA in CSF-PCR is helpful for diagnosis of primary central nervous system lymphoma, particularly in HIV/AIDS patients.

CT

Head CT scan may be helpful in the diagnosis of primary central nervous system lymphoma. Findings on CT scan suggestive of primary central nervous system lymphoma include supratentorial hyperattenuating, enhancing, hemorrhaging mass with subependymal extension crossing the corpus callosum. Chest, abdomen, and pelvic CT scans may be used to exclude any occult systemic disease from the spread of primary central nervous system lymphoma.

MRI

Contrast-enhanced MRI is the imaging modality of choice for primary central nervous system lymphoma. Findings on MRI suggestive of primary central nervous system lymphoma include solitary to multiple, 3-5 cm ring-enhancing lesions in almost any location, but usually deep in the white matter, which are typically hypointense on T1-weighted images and iso- to hypointense on T2-weighted images.

Other Imaging Findings

There are no other imaging findings associated with primary central nervous system lymphoma.

Other Diagnostic Studies

Other diagnostic studies for primary central nervous system lymphoma include magnetic resonance spectroscopy, magnetic resonance perfusion, scintigraphy, stereotactic biopsy, and polymerase chain reaction.

Treatment

Medical Therapy

The treatment of primary central nervous system lymphoma depends on the patient’s age, performance status, stage and location of the lymphoma, and whether or not the individual is immunosuppressed. Chemotherapy is the mainstay of treatment for primary central nervous system lymphoma. Corticosteroids are often used to destroy lymphoma cells and relieve cerebral edema. Radiation therapy may be given on its own or along with chemotherapy. The treatment of primary central nervous system lymphoma depends on the patient’s age, performance status, stage and location of the lymphoma, and whether or not the individual is immunosuppressed.

Surgery

Surgical intervention is not recommended for the management of primary central nervous system lymphoma, because the tumors are often deeply seated and spread throughout the brain.

Prevention

There are no primary or secondary preventive measures available for seminoma.

References


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Historical Perspective

Historical Perspective

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Overview

Historical Perspective

References


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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Primary CNS lymphomas are extranodal, malignant non-Hodgkin lymphomas of the diffuse large B-cell type that are confined to the brain, eyes, leptomeninges, or spinal cord, in the absence of systemic lymphoma. Primary CNS lymphomas are estimated to account for up to 1% of all lymphomas, 4–6% of all extranodal lymphomas, and about 3% of all CNS tumours. After a continuous rise in the incidence of primary CNS lymphoma during the 1980s and 1990s, epidemiological data in high-income countries show a decrease in incidence, particularly among young patients with AIDS.

Classification

Primary central nervous system lymphoma may be classified according to the site of involvement of the central nervous system into 3 groups:[1]

  • Parenchymal
  • Non-Hodgkin B cell type / diffuse large B-cell non-Hodgkin’s lymphoma (most common)
  • Non-Hodgkin T cell type
  • Primary CNS involvement with Hodgkin disease
  • Intravascular lymphoma
  • Primary leptomeningeal lymphoma
  • Primary dural lymphoma

References

  1. ↑ CNS lymphoma. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/cns-lymphoma-1. Accessed on February 17, 2016

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

Primary CNS lymphoma (PCNSL) is a highly aggressive non-Hodgkin lymphoma confined to the CNS, including the brain, spine, cerebrospinal fluid (CSF), and eyes. Unlike other brain tumors, it often has a favorable response to both chemotherapy and radiation therapy, but compared with lymphomas outside the CNS, survival is usually inferior. Moreover, the prognosis for PCNSL that has failed first-line therapy remains poor. Although new therapeutic approaches have improved survival, the management of this disease still poses a challenge in neuro-oncology. Primary central nervous system lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps.[1] EBV is almost always associated with primary CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it’s immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination.[2] Genes involved in the pathogenesis of primary central nervous system lymphoma include IG, BCL6, CD95, PAX5, and PIM1.[3] Primary central nervous system lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and iatrogenically immunosuppressed).[4] Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions).[5][2] On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage.[2] On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels. The atypical cells have large round, oval and angulated hyperchromatic and vesicular nuclei, and a narrow rim of pale cytoplasm. An occasional mitotic figure is noted.[4][6] Primary central nervous system lymphoma is demonstrated by positivity to tumor markers, including B lymphocyte markers, such as CD19, CD20, CD79a, Ki-67, GFAP, BCL-2, and BCL-6.[2][6][7][8] Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter. On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage. The lesion is mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia. Multiple lesions are reported in 38%–55% of non-AIDS primary CNS lymphomas. Multifocal intraparenchymal lesions, without a dural involvement, are very rare. Frontal lobe is affected in 20%–43% of primary CNS lymphoma. Other sites iclude brain stem, cerebellum, leptomeninges, spinal cord, and eyes. They may demonstrate areas of necrosis, especially in immunodeficient patients.

Pathogenesis

  • Primary central nervous system lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps.[1]
  • The understanding of the pathogenesis of primary central nervous system lymphoma is quite limited as the CNS is devoid of any B-cells.[2]
  • EBV is almost always associated with primary CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it’s immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination.[2]
  • Rubenstein et al demonstrated that interleukin 4 and STAT6 molecules are required for the retention of peripherally derived lymphoma cells in the central nervous system as well as for the angiocentric pattern in primary CNS lymphoma. Overexpression of STAT6 is also associated with tumor progression.[2]
  • Pathology reveals highly proliferative tumor cells in an angiocentric growth pattern, diffusely infiltrating the CNS.
  • Most PCNSLs are diffuse large B-cell lymphoma (DLBCL; 90%) and, rarely, Burkitt, low-grade, or T-cell lymphoma.
  • Gene-expression profiling has identified three molecular subgroups of non-CNS DLBCL, including the germinal center B-cell–like, activated B-cell–like, and type 3 subgroups.
  • Staining of PCNSL biopsies with antibodies that distinguish these DLBCL subgroups showed that the vast majority of PCNSLs were nongerminal center subtype.
  • the B-cell receptor signaling axis, with its downstream target, NFκB, is affected by frequent recurrent mutations, mainly in MYD88 and CD79B.

Genetics

  • Development of primary central nervous system lymphoma is the result of multiple genetic mutations.
  • Genes involved in the pathogenesis of primary central nervous system lymphoma include:[3]

Associated Conditions

Gross Pathology

  • Primary central nervous system lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions).[5][2]
  • On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage.[2]
  • The lesion is mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia.[9]
  • Multiple lesions are reported in 38%–55% of non-AIDS primary CNS lymphomas. Multifocal intraparenchymal lesions, without a dural involvement, are very rare. Frontal lobe is affected in 20%–43% of primary CNS lymphoma. Other sites iclude brain stem, cerebellum, leptomeninges, spinal cord, and eyes.
  • They may demonstrate areas of necrosis, especially in immunodeficient patients.
  • Origin of malignant cells is not well understood as intra-axial CNS does not have lymphatic system.[5]
  • Gross pathological specimen primary central nervous system lymphoma in the brain parenchyma. This autopsy photograph shows involvement of cerebral hemispheres by a primary CNS lymphoma in an AIDS patient. It was a large B-cell lymphoma and the patient succumbed to it within 6 months of diagnosis.[10]
    Gross pathological specimen primary central nervous system lymphoma in the brain parenchyma. This autopsy photograph shows involvement of cerebral hemispheres by a primary CNS lymphoma in an AIDS patient. It was a large B-cell lymphoma and the patient succumbed to it within 6 months of diagnosis.[10]

Microscopic Pathology

  • The vast majority (>90%) of primary central nervous system lymphoma are B-cell in origin: diffuse large B-cell lymphoma and high-grade Burkitt-like B-cell lymphoma.[5]
  • Malignant cells tend to accumulate around blood vessels (angiocentric pattern).[2]
  • Low-grade tumors are more frequently T-cell in origin.[5]
  • On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels. The atypical cells have large round, oval and angulated hyperchromatic and vesicular nuclei, and a narrow rim of pale cytoplasm. An occasional mitotic figure is noted.[4][6]
  • Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.[4]
    Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.[4]

Immunohistochemistry

Primary central nervous system lymphoma is demonstrated by positivity to tumor markers, including B lymphocyte markers, such as:[6][7][8][2]

  • The atypical cells of primary central nervous system lymphoma demonstrating strong surface immunostaining for the B lymphocyte markers, CD20.[6]
    The atypical cells of primary central nervous system lymphoma demonstrating strong surface immunostaining for the B lymphocyte markers, CD20.[6]

References

  1. ↑ 1.0 1.1 PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMAS IN THE NEUROLOGICAL PRACTICE. file:///C:/Users/Owner/Downloads/psyneur_2014_1_2_8.pdf. Accessed on February 19, 2016
  2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 Bhagavathi S, Wilson JD (2008). “Primary central nervous system lymphoma”. Arch Pathol Lab Med. 132 (11): 1830–4. doi:10.1043/1543-2165-132.11.1830. PMID 18976024.
  3. ↑ 3.0 3.1 Ferreri, A. J. M. (2011). “How I treat primary CNS lymphoma”. Blood. 118 (3): 510–522. doi:10.1182/blood-2011-03-321349. ISSN 0006-4971.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016
  5. ↑ 5.0 5.1 5.2 5.3 5.4 Pathology of primary central system lymphoma. Dr Amir Rezaee and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/primary-cns-lymphoma. Accessed on February 18, 2016
  6. ↑ 6.0 6.1 6.2 6.3 6.4 CNS lymphoma (primary). A.Prof Frank Gaillard. Radioapaedia 2016. http://radiopaedia.org/cases/cns-lymphoma-primary. Accessed on February 23, 2016
  7. ↑ 7.0 7.1 Haldorsen, Ingfrid S; Kråkenes, Jostein; Goplen, Anne K; Dunlop, Oona; Mella, Olav; Espeland, Ansgar (2008). “AIDS-related primary central nervous system lymphoma: a Norwegian national survey 1989–2003”. BMC Cancer. 8 (1): 225. doi:10.1186/1471-2407-8-225. ISSN 1471-2407.
  8. ↑ 8.0 8.1 Yamanaka, Ryuya (2013). “Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research”. doi:10.5772/52757.
  9. ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). “A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
  10. ↑ Image courtesy of Dr. A.Prof Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC


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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

There are no known direct causes for primary central nervous system lymphoma. To view a comprehensive list of risk factors that increase the risk of primary nervous system lymphoma, click here.

Causes

  • There are no known direct causes for primary central nervous system lymphoma.
  • Common risk factors for primary central nervous system lymphoma can be found here.[1][2][3]

References

  1. ↑ Risks of primary central nervous lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016
  2. ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). “A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
  3. ↑ Epidemiology of primary CNS lymphoma. Dr Amir Rezaee and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/primary-cns-lymphoma. Accessed on February 18, 2016


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Differentiating Primary Central Nervous System Lymphoma from other Diseases

Differentiating Primary Central Nervous System Lymphoma from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.

Overview

On the basis of seizure, visual disturbance, and constitutional symptoms, astrocytoma must be differentiated from oligodendroglioma, meningioma, hemangioblastoma, pituitary adenoma, schwannoma, primary CNS lymphoma, medulloblastoma, ependymoma, craniopharyngioma, pinealoma, AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.

Differentiating primary central nervous system lymphoma from other Diseases

Differentiating primary central nervous system lymphomafrom other diseases on the basis of seizure, visual disturbance, and constitutional symptoms

On the basis of seizure, visual disturbance, and constitutional symptoms, primary central nervous system lymphomamust be differentiated from oligodendroglioma, meningioma, hemangioblastoma, pituitary adenoma, schwannoma, astrocytoma, medulloblastoma, ependymoma, craniopharyngioma, pinealoma, AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.

Diseases Clinical manifestations Para-clinical findings Gold
standard 		Additional findings
Symptoms Physical examination
Lab Findings MRI Immunohistopathology
Head-
ache 		Seizure Visual disturbance Constitutional Focal neurological deficit
Adult primary brain tumors
Primary CNS lymphoma
[1][2] 		+ +/− +/− − + −
  • Single mass with ring enhancement
    Glioblastoma multiforme
    [3][4][5]     		+ +/− +/− − + −
    • Pseudopalisading appearance
    Oligodendroglioma
    [6][7][8]     		+ + +/− − + −
    • Chicken wire capillary pattern
    • Fried egg cell appearance
    Meningioma
    [9][10][11]     		+ +/− +/− − + −
    • Well circumscribed
    • Extra-axial mass
    • Whorled spindle cell pattern
    • May be associated with NF-2
    Hemangioblastoma
    [12][13][14][15]     		+ +/− +/− − + −
    Pituitary adenoma
    [16][17][5]     		− − + Bitemporal hemianopia − −
    • It is associated with MEN1 disease.
        Schwannoma
        [18][19][20][21]         		− − − − + −
        • Split-fat sign
        • Fascicular sign
        • Often have areas of hemosiderin
        • S100+
        Childhood primary brain tumors
        Pilocytic astrocytoma
        [22][23][24]         		+ +/− +/− − + −
        Medulloblastoma
        [25][26][27]         		+ +/− +/− − + −
        • Homer wright rosettes
        Ependymoma
        [28][5]         		+ +/− +/− − + −
        • Hydrocephalus
        • Causes an unusually persistent, continuous headache in children.
        Craniopharyngioma
        [29][30][31][5]         		+ +/− + Bitemporal hemianopia − +
        Pinealoma
        [32][33][34]         		+ +/− +/− − + vertical gaze palsy
        • May cause prinaud syndrome (vertical gaze palsy, pupillary light-near dissociation, lid retraction and convergence-retraction nystagmus
        Vascular
        AV malformation
        [35][36][5]         		+ + +/− − +/− −
        Brain aneurysm
        [37][38][39][40][41]         		+ +/− +/− − +/− −
        • MRA and CTA
        Infectious
        Bacterial brain abscess
        [42][43]         		+ +/− +/− + +
        • Central hypodense signal and surrounding ring-enhancement in T1
        • Central hyperintense area surrounded by a well-defined hypointense capsule with surrounding edema in T2
        • History/ imaging
        Tuberculosis
        [44][5][45]         		+ +/− +/− + +
        • Lab data/ Imaging
        Toxoplasmosis
        [46][47]         		+ +/− +/− − +
        • History/ imaging
        Hydatid cyst
        [48][5]         		+ +/− +/− +/− +
        • Imaging
        CNS cryptococcosis
        [49]         		+ +/− +/− + +
        • We may see numerous acutely branching septate hyphae
        • Lab data/ Imaging
        CNS aspergillosis
        [50]         		+ +/− +/− + +
        • Multiple abscesses
        • Ring enhancement
        • Peripheral low signal intensity on T2
        • We may see numerous acutely branching septate hyphae
        • Lab data/ Imaging
        Other
        Brain metastasis
        [51][5]         		+ +/− +/− + + −
        • Based on the primary cancer type we may have different immunohistopathology findings.
        • History/ imaging

        ABBREVIATIONS

        CNS=Central nervous system, AV=Arteriovenous, CSF=Cerebrospinal fluid, NF-2=Neurofibromatosis type 2, MEN-1=Multiple endocrine neoplasia, GFAP=Glial fibrillary acidic protein, HIV=Human immunodeficiency virus, BhCG=Human chorionic gonadotropin, ESR=Erythrocyte sedimentation rate, AFB=Acid fast bacilli, MRA=Magnetic resonance angiography, CTA=CT angiography

        References

        1. ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
        2. ↑ Paulus, Werner (1999). “Classification, Pathogenesis and Molecular Pathology of Primary CNS Lymphomas”. Journal of Neuro-Oncology. 43 (3): 203–208. doi:10.1023/A:1006242116122. ISSN 0167-594X.
        3. ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
        4. ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
        5. ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 5.7 Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
        6. ↑ Smits M (2016). “Imaging of oligodendroglioma”. Br J Radiol. 89 (1060): 20150857. doi:10.1259/bjr.20150857. PMC 4846213. PMID 26849038.
        7. ↑ Wesseling P, van den Bent M, Perry A (June 2015). “Oligodendroglioma: pathology, molecular mechanisms and markers”. Acta Neuropathol. 129 (6): 809–27. doi:10.1007/s00401-015-1424-1. PMC 4436696. PMID 25943885.
        8. ↑ Kerkhof M, Benit C, Duran-Pena A, Vecht CJ (2015). “Seizures in oligodendroglial tumors”. CNS Oncol. 4 (5): 347–56. doi:10.2217/cns.15.29. PMC 6082346. PMID 26478444.
        9. ↑ Zee CS, Chin T, Segall HD, Destian S, Ahmadi J (June 1992). “Magnetic resonance imaging of meningiomas”. Semin. Ultrasound CT MR. 13 (3): 154–69. PMID 1642904.
        10. ↑ Shibuya M (2015). “Pathology and molecular genetics of meningioma: recent advances”. Neurol. Med. Chir. (Tokyo). 55 (1): 14–27. doi:10.2176/nmc.ra.2014-0233. PMID 25744347.
        11. ↑ Begnami MD, Palau M, Rushing EJ, Santi M, Quezado M (September 2007). “Evaluation of NF2 gene deletion in sporadic schwannomas, meningiomas, and ependymomas by chromogenic in situ hybridization”. Hum. Pathol. 38 (9): 1345–50. doi:10.1016/j.humpath.2007.01.027. PMC 2094208. PMID 17509660.
        12. ↑ Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH (May 2014). “Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease”. J. Neurosurg. 120 (5): 1055–62. doi:10.3171/2014.1.JNS131431. PMC 4762041. PMID 24579662.
        13. ↑ Hussein MR (October 2007). “Central nervous system capillary haemangioblastoma: the pathologist’s viewpoint”. Int J Exp Pathol. 88 (5): 311–24. doi:10.1111/j.1365-2613.2007.00535.x. PMC 2517334. PMID 17877533.
        14. ↑ Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH (May 1989). “Posterior fossa hemangioblastomas: MR imaging”. Radiology. 171 (2): 463–8. doi:10.1148/radiology.171.2.2704812. PMID 2704812.
        15. ↑ Perks WH, Cross JN, Sivapragasam S, Johnson P (March 1976). “Supratentorial haemangioblastoma with polycythaemia”. J. Neurol. Neurosurg. Psychiatry. 39 (3): 218–20. PMID 945331.
        16. ↑ Kucharczyk W, Davis DO, Kelly WM, Sze G, Norman D, Newton TH (December 1986). “Pituitary adenomas: high-resolution MR imaging at 1.5 T”. Radiology. 161 (3): 761–5. doi:10.1148/radiology.161.3.3786729. PMID 3786729.
        17. ↑ Syro LV, Scheithauer BW, Kovacs K, Toledo RA, Londoño FJ, Ortiz LD, Rotondo F, Horvath E, Uribe H (2012). “Pituitary tumors in patients with MEN1 syndrome”. Clinics (Sao Paulo). 67 Suppl 1: 43–8. PMC 3328811. PMID 22584705.
        18. ↑ Donnelly, Martin J.; Daly, Carmel A.; Briggs, Robert J. S. (2007). “MR imaging features of an intracochlear acoustic schwannoma”. The Journal of Laryngology & Otology. 108 (12). doi:10.1017/S0022215100129056. ISSN 0022-2151.
        19. ↑ Feany MB, Anthony DC, Fletcher CD (May 1998). “Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge”. Histopathology. 32 (5): 405–10. PMID 9639114.
        20. ↑ Chen H, Xue L, Wang H, Wang Z, Wu H (July 2017). “Differential NF2 Gene Status in Sporadic Vestibular Schwannomas and its Prognostic Impact on Tumour Growth Patterns”. Sci Rep. 7 (1): 5470. doi:10.1038/s41598-017-05769-0. PMID 28710469.
        21. ↑ Hardell, Lennart; Hansson Mild, Kjell; Sandström, Monica; Carlberg, Michael; Hallquist, Arne; Påhlson, Anneli (2003). “Vestibular Schwannoma, Tinnitus and Cellular Telephones”. Neuroepidemiology. 22 (2): 124–129. doi:10.1159/000068745. ISSN 0251-5350.
        22. ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
        23. ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
        24. ↑ Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
        25. ↑ Dorwart, R H; Wara, W M; Norman, D; Levin, V A (1981). “Complete myelographic evaluation of spinal metastases from medulloblastoma”. Radiology. 139 (2): 403–408. doi:10.1148/radiology.139.2.7220886. ISSN 0033-8419.
        26. ↑ Fruehwald-Pallamar, Julia; Puchner, Stefan B.; Rossi, Andrea; Garre, Maria L.; Cama, Armando; Koelblinger, Claus; Osborn, Anne G.; Thurnher, Majda M. (2011). “Magnetic resonance imaging spectrum of medulloblastoma”. Neuroradiology. 53 (6): 387–396. doi:10.1007/s00234-010-0829-8. ISSN 0028-3940.
        27. ↑ Burger, P. C.; Grahmann, F. C.; Bliestle, A.; Kleihues, P. (1987). “Differentiation in the medulloblastoma”. Acta Neuropathologica. 73 (2): 115–123. doi:10.1007/BF00693776. ISSN 0001-6322.
        28. ↑ Yuh, E. L.; Barkovich, A. J.; Gupta, N. (2009). “Imaging of ependymomas: MRI and CT”. Child’s Nervous System. 25 (10): 1203–1213. doi:10.1007/s00381-009-0878-7. ISSN 0256-7040.
        29. ↑ Brunel H, Raybaud C, Peretti-Viton P, Lena G, Girard N, Paz-Paredes A, Levrier O, Farnarier P, Manera L, Choux M (September 2002). “[Craniopharyngioma in children: MRI study of 43 cases]”. Neurochirurgie (in French). 48 (4): 309–18. PMID 12407316.
        30. ↑ Prabhu, Vikram C.; Brown, Henry G. (2005). “The pathogenesis of craniopharyngiomas”. Child’s Nervous System. 21 (8–9): 622–627. doi:10.1007/s00381-005-1190-9. ISSN 0256-7040.
        31. ↑ Kennedy HB, Smith RJ (December 1975). “Eye signs in craniopharyngioma”. Br J Ophthalmol. 59 (12): 689–95. PMC 1017436. PMID 766825.
        32. ↑ Ahmed SR, Shalet SM, Price DA, Pearson D (September 1983). “Human chorionic gonadotrophin secreting pineal germinoma and precocious puberty”. Arch. Dis. Child. 58 (9): 743–5. PMID 6625640.
        33. ↑ Sano, Keiji (1976). “Pinealoma in Children”. Pediatric Neurosurgery. 2 (1): 67–72. doi:10.1159/000119602. ISSN 1016-2291.
        34. ↑ Baggenstoss, Archie H. (1939). “PINEALOMAS”. Archives of Neurology And Psychiatry. 41 (6): 1187. doi:10.1001/archneurpsyc.1939.02270180115011. ISSN 0096-6754.
        35. ↑ Kucharczyk, W; Lemme-Pleghos, L; Uske, A; Brant-Zawadzki, M; Dooms, G; Norman, D (1985). “Intracranial vascular malformations: MR and CT imaging”. Radiology. 156 (2): 383–389. doi:10.1148/radiology.156.2.4011900. ISSN 0033-8419.
        36. ↑ Fleetwood, Ian G; Steinberg, Gary K (2002). “Arteriovenous malformations”. The Lancet. 359 (9309): 863–873. doi:10.1016/S0140-6736(02)07946-1. ISSN 0140-6736.
        37. ↑ Chapman, Arlene B.; Rubinstein, David; Hughes, Richard; Stears, John C.; Earnest, Michael P.; Johnson, Ann M.; Gabow, Patricia A.; Kaehny, William D. (1992). “Intracranial Aneurysms in Autosomal Dominant Polycystic Kidney Disease”. New England Journal of Medicine. 327 (13): 916–920. doi:10.1056/NEJM199209243271303. ISSN 0028-4793.
        38. ↑ Castori M, Voermans NC (October 2014). “Neurological manifestations of Ehlers-Danlos syndrome(s): A review”. Iran J Neurol. 13 (4): 190–208. PMC 4300794. PMID 25632331.
        39. ↑ Schievink, W. I.; Raissi, S. S.; Maya, M. M.; Velebir, A. (2010). “Screening for intracranial aneurysms in patients with bicuspid aortic valve”. Neurology. 74 (18): 1430–1433. doi:10.1212/WNL.0b013e3181dc1acf. ISSN 0028-3878.
        40. ↑ Germain DP (May 2017). “Pseudoxanthoma elasticum”. Orphanet J Rare Dis. 12 (1): 85. doi:10.1186/s13023-017-0639-8. PMC 5424392. PMID 28486967.
        41. ↑ Farahmand M, Farahangiz S, Yadollahi M (October 2013). “Diagnostic Accuracy of Magnetic Resonance Angiography for Detection of Intracranial Aneurysms in Patients with Acute Subarachnoid Hemorrhage; A Comparison to Digital Subtraction Angiography”. Bull Emerg Trauma. 1 (4): 147–51. PMC 4789449. PMID 27162847.
        42. ↑ Haimes, AB; Zimmerman, RD; Morgello, S; Weingarten, K; Becker, RD; Jennis, R; Deck, MD (1989). “MR imaging of brain abscesses”. American Journal of Roentgenology. 152 (5): 1073–1085. doi:10.2214/ajr.152.5.1073. ISSN 0361-803X.
        43. ↑ Brouwer, Matthijs C.; Tunkel, Allan R.; McKhann, Guy M.; van de Beek, Diederik (2014). “Brain Abscess”. New England Journal of Medicine. 371 (5): 447–456. doi:10.1056/NEJMra1301635. ISSN 0028-4793.
        44. ↑ Morgado, Carlos; Ruivo, Nuno (2005). “Imaging meningo-encephalic tuberculosis”. European Journal of Radiology. 55 (2): 188–192. doi:10.1016/j.ejrad.2005.04.017. ISSN 0720-048X.
        45. ↑ Be NA, Kim KS, Bishai WR, Jain SK (March 2009). “Pathogenesis of central nervous system tuberculosis”. Curr. Mol. Med. 9 (2): 94–9. PMC 4486069. PMID 19275620.
        46. ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
        47. ↑ Helton KJ, Maron G, Mamcarz E, Leventaki V, Patay Z, Sadighi Z (November 2016). “Unusual magnetic resonance imaging presentation of post-BMT cerebral toxoplasmosis masquerading as meningoencephalitis and ventriculitis”. Bone Marrow Transplant. 51 (11): 1533–1536. doi:10.1038/bmt.2016.168. PMID 27348541.
        48. ↑ Taslakian B, Darwish H (September 2016). “Intracranial hydatid cyst: imaging findings of a rare disease”. BMJ Case Rep. 2016. doi:10.1136/bcr-2016-216570. PMC 5030532. PMID 27620198.
        49. ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
        50. ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
        51. ↑ Pope WB (2018). “Brain metastases: neuroimaging”. Handb Clin Neurol. 149: 89–112. doi:10.1016/B978-0-12-811161-1.00007-4. PMC 6118134. PMID 29307364.

        Template:WH Template:WS

        Epidemiology and Demographics

        Epidemiology and Demographics

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

        Overview

        Primary CNS lymphoma can develop in immunosuppressed (HIV/AIDS, organ transplant, immunosuppressive agents) or immunocompetent patients. Primary central nervous system lymphoma constitutes approximately 3%-4% of all primary brain tumors and 1%-2% of all lymphomas. Primary central nervous system lymphoma has been diagnosed in at least 2% of individuals infected with human immunodeficiency virus, and in 9–14% of acquired immunodeficiency syndrome (AIDS)-autopsies. Primary CNS lymphoma is, after toxoplasmosis, the most common cause of focal brain lesions in AIDS patients. The overall incidence of primary central nervous system lymphoma is 0.4 per 100,000 individuals per year. The median age of occurrence of primary central nervous system lymphoma in immunocompetent and immunocompromised patients are 53-57 years and 31-35 years, respectively. The peak incidence is between 60 and 70 years old for immunocompetent patients. Significant increment of incidence rate over time is associated with increased incidence of AIDS and advanced age. Males are more commonly affected with primary central nervous system lymphoma than females. The male to female ratio is approximately 1.2 to 1 in immunocompetent patients and approximately 7.38 to 1 in AIDS-associated primary central nervous system lymphoma.

        Epidemiology and Demographics

        Prevalence

        • Primary central nervous system lymphoma constitutes approximately 3%-4% of all primary brain tumors.[1]
        • Primary CNS lymphoma constitues approximately 1%-2% of all lymphomas.[1]
        • Primary central nervous system lymphoma has been diagnosed in at least 2% of individuals infected with human immunodeficiency virus, and in 9–14% of acquired immunodeficiency syndrome (AIDS)-autopsies.[2]
        • Primary CNS lymphoma is, after toxoplasmosis, the most common cause of focal brain lesions in AIDS patients.[2]

        Incidence

        • The overall incidence rate of primary central nervous system lymphoma is 0.4 cases per 100,000 individuals per year.[1]

        Age

        • Primary central nervous system lymphoma does not have predilections for any age group.[3]
        • The median age of occurrence of primary central nervous system lymphoma in immunocompetent and immunocompromised patients are 53-57 years and 31-35 years, respectively.[4]
        • The peak incidence is between 60 and 70 years old for immunocompetent patients.[1]
        • Young age of the patient should be noted, because it is not typical for primary CNS lymphoma.
        • Significant increment of incidence rate over time is associated with increased incidence of AIDS and advanced age.

        Gender

        • Males are more commonly affected with primary central nervous system lymphoma than females.[1]
        • The male to female ratio is approximately 1.2 to 1 in immunocompetent patients and approximately 7.38 to 1 in AIDS-associated primary central nervous system lymphoma.[4]

        References

        1. ↑ 1.0 1.1 1.2 1.3 1.4 Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). “A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
        2. ↑ 2.0 2.1 Haldorsen, Ingfrid S; Kråkenes, Jostein; Goplen, Anne K; Dunlop, Oona; Mella, Olav; Espeland, Ansgar (2008). “AIDS-related primary central nervous system lymphoma: a Norwegian national survey 1989–2003”. BMC Cancer. 8 (1): 225. doi:10.1186/1471-2407-8-225. ISSN 1471-2407.
        3. ↑ Primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016
        4. ↑ 4.0 4.1 Bhagavathi S, Wilson JD (2008). “Primary central nervous system lymphoma”. Arch Pathol Lab Med. 132 (11): 1830–4. doi:10.1043/1543-2165-132.11.1830. PMID 18976024.


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        Risk Factors

        Risk Factors

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

        Overview

        The most potent risk factor in the development of primary central nervous system lymphoma is a weakened or suppressed immune system in individuals who have acquired immunodeficiency syndrome (AIDS), received an organ transplant and are on immunosuppressants, inherited immunosuppressive disorders (IgA deficiency, Wiskott-Aldrich syndrome, ataxia telangiectasia), and autoimmune disorders (systemic lupus erythematosus, rheumatoid arthritis, myasthenia gravis).

        Risk Factors

        The most potent risk factor in the development of primary central nervous system lymphoma is a weakened or suppressed immune system in individuals who have:[1][2][3][4]

        • The highest number of cases occur in individuals with AIDS.
        • Received an organ transplant and are on immunosuppressants
        • Inherited immunosuppressive disorder
        • Autoimmune disorders

        References

        1. ↑ Risks of primary central nervous lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016
        2. ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). “A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
        3. ↑ Epidemiology of primary CNS lymphoma. Dr Amir Rezaee and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/primary-cns-lymphoma. Accessed on February 18, 2016
        4. ↑ Bhagavathi S, Wilson JD (2008). “Primary central nervous system lymphoma”. Arch Pathol Lab Med. 132 (11): 1830–4. doi:10.1043/1543-2165-132.11.1830. PMID 18976024.


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        Screening

        Screening

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

        Overview

        There is insufficient evidence to recommend routine screening for primary central nervous system lymphoma.[1]

        Screening

        There is insufficient evidence to recommend routine screening for primary central nervous system lymphoma.[1]

        References

        1. ↑ 1.0 1.1 Screening of primary central nervous system lymphoma. U.S. preventive services task force 2016. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=PRIMARY+CENTRAL+NERVOUS+SYSTEM+LYMPHOMA. Accessed on February 24, 2016


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        Natural History, Complications and Prognosis

        Natural History, Complications and Prognosis

        Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

        Overview

        If left untreated, primary central nervous system lymphoma may progress to develop elevated intracranial pressure, ocular symptoms, focal neurological deficits, and neuropsychiatric problems. Common complications of primary central nervous system lymphoma include relapse, extracranial or subcutaneous metastasis, neuropsychiatric problems, and neurological toxicity. The prognosis of primary central nervous system lymphoma is generally poor.

        Natural History

        • Primary central nervous system lymphoma is usually an aggressive lymphoma.[1]
        • The clinical course is rapidly fatal; these patients usually present with signs of elevated intracranial pressure, nausea, papilledema, vomiting, and neuropsychiatric symptoms.[2]

        Complications

        Common complications of primary central nervous system lymphoma include:[3][4][5]

        • Neurological toxicity

        Neurological Toxicity

        Prognosis

        • The prognosis of primary central nervous system lymphoma is generally poor.[1][4]
        • Poor prognostic factors for primary central nervous system lymphoma include:[1][6]

        In Immunocompetent Patients

        In AIDS patients

        References

        1. ↑ 1.0 1.1 1.2 Prognosis of primary central nervous system lymphoma. Canadian cancer society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-cns-lymphoma/?region=on. Accessed on February 17, 2016
        2. ↑ Manenti, G.; Di Giuliano, F.; Bindi, A.; Liberto, V.; Funel, V.; Garaci, F. G.; Floris, R.; Simonetti, G. (2013). “A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/916348. ISSN 2090-6862.
        3. ↑ Symptoms of primary CNS Lymphoma. Lymphomation 2016. http://www.lymphomation.org/type-cns.htm. Accessed on February 24, 2016
        4. ↑ 4.0 4.1 Ahmed, Zartaj; Ramanathan, Ramesh K.; Ram, Sunil; Newell, James; Halepota, Maqbool (2014). “Unusual Relapse of Primary Central Nervous System Lymphoma at Site of Lumbar Puncture”. Case Reports in Hematology. 2014: 1–4. doi:10.1155/2014/161952. ISSN 2090-6560.
        5. ↑ 5.0 5.1 5.2 5.3 5.4 Yamanaka, Ryuya (2013). “Primary Central Nervous System Lymphoma − Recent Advance on Clinical Research”. doi:10.5772/52757.
        6. ↑ Prognsotic factors for primary CNS lymphoma. National cancer institute 2016. http://www.cancer.gov/types/lymphoma/hp/primary-cns-lymphoma-treatment-pdq. Accessed on February 19, 2016
        7. ↑ 7.0 7.1 7.2 Prognosis of primary central nervous system lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Primary_central_nervous_system_lymphoma. Accessed on February 18, 2016


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        Diagnosis

        Diagnosis

        Staging | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

        Treatment

        Treatment

        Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

        Case Studies

        Case Studies

        Case #1


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