PCNSL
MCID: PRM226
MIFTS: 47

Primary Central Nervous System Lymphoma (PCNSL)

Categories: Blood diseases, Cancer diseases, Eye diseases, Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Primary Central Nervous System Lymphoma

MalaCards integrated aliases for Primary Central Nervous System Lymphoma:

Name: Primary Central Nervous System Lymphoma 20 58 36 54 70
Primary Brain Lymphoma 20 58
Primary Cns Lymphoma 20 58
Pcnsl 20 58
Primary Cerebral Lymphoma 70
Primary Lymphoma, Cns 20

Characteristics:

Orphanet epidemiological data:

58
primary central nervous system lymphoma
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare haematological diseases


External Ids:

KEGG 36 H02424
ICD10 via Orphanet 33 C83.3
UMLS via Orphanet 71 C0240803 C0280803
Orphanet 58 ORPHA46135
UMLS 70 C0240803 C0280803

Summaries for Primary Central Nervous System Lymphoma

GARD : 20 Primary central nervous system lymphoma (primary CNS lymphoma) is a rare form of non-Hodgkin lymphoma in which cancerous cells develop in the lymph tissue of the brain and/or spinal cord. Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma). The signs and symptoms vary based on which parts of the central nervous system are affected, but may include nausea and vomiting; seizures ; headaches; arm or leg weakness; confusion; double vision and/or hearing loss. The exact underlying cause of primary CNS lymphoma is poorly understood; however, people with a weakened immune system (such as those with acquired immunodeficiency syndrome ) or who have had an organ transplant appear to have an increased risk of developing the condition. Treatment varies based on the severity of the condition and location of the cancerous cells.

MalaCards based summary : Primary Central Nervous System Lymphoma, also known as primary brain lymphoma, is related to intraocular lymphoma and central nervous system lymphoma. An important gene associated with Primary Central Nervous System Lymphoma is MYD88 (MYD88 Innate Immune Signal Transduction Adaptor), and among its related pathways/superpathways are B cell receptor signaling pathway and NF-kappaB Signaling. The drugs Teniposide and Pemetrexed have been mentioned in the context of this disorder. Affiliated tissues include spinal cord, eye and brain, and related phenotypes are cellular and hematopoietic system

KEGG : 36 Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. Most PCNSLs are diffuse large B-cell lymphoma (DLBCL; 90%), with the remainder consisting of T-cell lymphomas, poorly characterized low-grade lymphomas, or Burkitt lymphomas. The molecular pathogenesis of PCNSL is now better understood. The B-cell receptor (BCR) signaling axis with its downstream target, NF-kappa B, is affected by frequent recurrent mutations, mainly in MYD88, CD79B and, less frequently, CARD11, and TNFAIP3. Recurring chromosomal losses affect the 6q, 6p21.32 (HLA locus) and 9p21 (CDKN2A locus) regions.

Wikipedia : 73 A primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of... more...

Related Diseases for Primary Central Nervous System Lymphoma

Diseases in the Central Nervous System Lymphoma family:

Primary Central Nervous System Lymphoma

Diseases related to Primary Central Nervous System Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 373)
# Related Disease Score Top Affiliating Genes
1 intraocular lymphoma 31.6 PAX5 CXCR5 BCL6 BCL2
2 central nervous system lymphoma 31.5 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
3 lymphoma, hodgkin, classic 31.4 PAX5 BCL6 BCL2
4 diffuse large b-cell lymphoma 31.3 PAX5 MYD88 BCL6 BCL2
5 lymphoma, non-hodgkin, familial 30.8 PAX5 MYD88 CXCR5 BCL6 BCL2
6 lymphosarcoma 30.8 BCL6 BCL2
7 intravascular large b-cell lymphoma 30.4 MYD88 BCL6 BCL2
8 lymphoma 30.3 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
9 human herpesvirus 8 30.3 BCL6 BCL2
10 b-cell lymphoma 30.3 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
11 retina lymphoma 30.2 CXCR5 CXCL13
12 lymphoplasmacytic lymphoma 30.0 PAX5 MYD88 BCL6
13 reticulosarcoma 30.0 PAX5 BCL6 BCL2
14 marginal zone b-cell lymphoma 29.9 PAX5 MYD88 BCL6 BCL2
15 burkitt lymphoma 29.9 PAX5 CXCR5 CXCL13 BCL6 BCL2
16 b-lymphoblastic leukemia/lymphoma 29.8 PAX5 CXCR5 CXCL13
17 angioimmunoblastic t-cell lymphoma 29.8 CXCL13 BCL6
18 follicular lymphoma 29.6 IGHM CXCR5 BCL6 BCL2
19 leukemia, chronic lymphocytic 29.1 PAX5 MYD88 IGHM CXCR5 BCL6 BCL2
20 lymphoma, mucosa-associated lymphoid type 29.0 PAX5 MYD88 CXCR5 CXCL13 BCL6 BCL2
21 acquired immunodeficiency syndrome 10.9
22 glioblastoma 10.9
23 high grade glioma 10.6
24 toxoplasmosis 10.6
25 rare tumor 10.6
26 immune deficiency disease 10.6
27 glioma 10.6
28 glial tumor 10.6
29 neutropenia 10.6
30 uveitis 10.6
31 human immunodeficiency virus type 1 10.5
32 hydrocephalus 10.5
33 ataxia and polyneuropathy, adult-onset 10.5
34 lymphoproliferative syndrome 10.5
35 intracranial hypertension 10.5
36 parkinsonism 10.4
37 lupus erythematosus 10.4
38 encephalitis 10.4
39 systemic lupus erythematosus 10.4
40 cerebral lymphoma 10.4
41 progressive multifocal leukoencephalopathy 10.4
42 diabetes insipidus 10.4
43 encephalopathy 10.4
44 pars planitis 10.4
45 meningioma, familial 10.4
46 anaplastic large cell lymphoma 10.4
47 mucositis 10.4
48 intracranial meningioma 10.4
49 secretory meningioma 10.4
50 lymphoplasmacyte-rich meningioma 10.4

Graphical network of the top 20 diseases related to Primary Central Nervous System Lymphoma:



Diseases related to Primary Central Nervous System Lymphoma

Symptoms & Phenotypes for Primary Central Nervous System Lymphoma

MGI Mouse Phenotypes related to Primary Central Nervous System Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.7 BCL2 BCL6 CXCL13 CXCR5 MYD88 PAX5
2 hematopoietic system MP:0005397 9.5 BCL2 BCL6 CXCL13 CXCR5 MYD88 PAX5
3 immune system MP:0005387 9.17 BCL2 BCL6 CXCL13 CXCR5 MYD88 PAX5

Drugs & Therapeutics for Primary Central Nervous System Lymphoma

Drugs for Primary Central Nervous System Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 138)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Teniposide Approved Phase 4 29767-20-2 34698
2
Pemetrexed Approved, Investigational Phase 4 150399-23-8, 137281-23-3 60843 446556
3
Fotemustine Investigational Phase 4 92118-27-9
4 Formoterol Fumarate Phase 4
5
Ifosfamide Approved Phase 3 3778-73-2 3690
6
Busulfan Approved, Investigational Phase 3 55-98-1 2478
7
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
8
Dalteparin Approved Phase 3 9005-49-6
9
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
10
Isophosphamide mustard Phase 3 100427
11 Anticoagulants Phase 3
12 Fibrinolytic Agents Phase 3
13 Heparin, Low-Molecular-Weight Phase 3
14
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
15
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
16
tannic acid Approved Phase 1, Phase 2 1401-55-4
17
Benzocaine Approved, Investigational Phase 1, Phase 2 1994-09-7, 94-09-7 2337
18
Vindesine Approved, Investigational Phase 2 59917-39-4, 53643-48-4 40839
19
Nedaplatin Approved, Investigational Phase 2 95734-82-0
20
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
21
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
22
Bortezomib Approved, Investigational Phase 1, Phase 2 179324-69-7 93860 387447
23
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
24
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
25
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
26
Carmustine Approved, Investigational Phase 2 154-93-8 2578
27
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
28
Ganciclovir Approved, Investigational Phase 2 82410-32-0 3454
29
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4
30
Melphalan Approved Phase 2 148-82-3 4053 460612
31
Sargramostim Approved, Investigational Phase 2 123774-72-1, 83869-56-1
32
Mesna Approved, Investigational Phase 2 3375-50-6 598
33
Cobalt Approved, Experimental Phase 2 7440-48-4 104729
34
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
35
Mannitol Approved, Investigational Phase 1, Phase 2 69-65-8 6251 453
36
nivolumab Approved Phase 2 946414-94-4
37
Pembrolizumab Approved Phase 2 1374853-91-4
38
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
39
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
40
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
41
Etoposide Approved Phase 2 33419-42-0 36462
42
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
43
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
44
Idarubicin Approved Phase 2 58957-92-9 42890
45
leucovorin Approved Phase 2 58-05-9 6006
46
rituximab Approved Phase 2 174722-31-7 10201696
47
Zidovudine Approved Phase 2 30516-87-1 35370
48
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
49
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
50
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755

Interventional clinical trials:

(show top 50) (show all 126)
# Name Status NCT ID Phase Drugs
1 The Prospective Study of FVD Program and HD-MTX-Ara-C Program Contrast in the Treatment of PCNSL Lymphoma. Unknown status NCT01960192 Phase 4 HD-MTX-Ara-C regimen;FVD regimen
2 Phase IV Study on the Role of Whole Brain Irradiation in Primary CNS Lymphoma (PCNSL) After High-dose Methotrexate Completed NCT00153530 Phase 4 methotrexate
3 Open Randomized Prospective Clinical Study of Rituximab Combined With Fotemustine, Pemetrexed, Dexamethasone Versus Rituximab Plus Methotrexate, Cytarabine, and Dexamethasone in the Treatment of Primary Central Nervous System Lymphoma Recruiting NCT04083066 Phase 4 rituximab in combination with methotrexate, cytarabine and dexamethasone
4 A Prospective Clinical Study of a New Generation of BTK Inhibitors Combined With PD-1 and Formustine in the First-line Treatment of Primary Central Nervous System Lymphoma Recruiting NCT04831658 Phase 4 Orelabrutinib combined with PD-1 and fotemustine
5 High-dose Chemotherapy and Autologous Stem Cell Transplant or Consolidating Conventional Chemotherapy in Primary CNS Lymphoma - Randomized Phase III Trial Unknown status NCT02531841 Phase 3 Arm A (Fortecortin®-ETOPOPHOS®-IFO-cell®-CARBO-cell®);Arm B (TEPADINA®-CARMUBRIS®-Busilvex®)
6 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
7 Therapeutic Effects of R-IDARAM and Intrathecal Immunochemotherapy on Elderly Patients With Primary CNS Lymphoma Recruiting NCT02836158 Phase 2, Phase 3 R-IDARAM plus intrathecal chemotherapy
8 Treatment of Primary CNS Lymphoma With Systemic R-IDARAM Chemotherapy and Intrathecal Immunochemotherapy Recruiting NCT02657785 Phase 2, Phase 3 R-IDARAM plus intrathecal chemotherapy
9 Phase III Trial Evaluating Maintenance Treatment Versus Observation in Elderly Patients Suffering From Primary Central Nervous System Lymphoma in Complete Remission After High Dose Methotrexate Based Chemotherapy in First Line Recruiting NCT02313389 Phase 3 Rituximab, Methotrexate, Temozolomide
10 Busulfan+ Cyclophosphamide+ Etoposide Conditioning Regimen for Primary Central Nervous System Lymphoma Undergoing Autologous Hematopoietic Stem Cell Transplantation Recruiting NCT03733327 Phase 2, Phase 3 Busulfan (BU);Cyclophosphamide (CY);Etoposide (VP-16)
11 Temozolomide in Treatment of Patients With Primary Central Nervous System Lymphoma Unknown status NCT02934204 Phase 2 methotrexate;temozolomide
12 Open-label Single Arm Phase II Study on Pembrolizumab for Recurrent Primary Central Nervous System Lymphoma (PCNSL) Unknown status NCT02779101 Phase 2 pembrolizumab
13 Plasma and Cerebrospinal Fluid (CSF) Pharmacokinetics of Bendamustine as a Component of Salvage Therapy for Primary Central Nervous System Lymphoma (PCNSL) Unknown status NCT03392714 Phase 2 R-B(O)AD
14 Treatment of HIV-Related Primary Central Nervous System Lymphoma (HIV-PCNSL): A Phase II Trial With Neoadjuvant Chemotherapy (High-Dose Methotrexate (MTX) Plus High-Dose Zidovudine) and Radiotherapy Unknown status NCT00003261 Phase 2 methotrexate;zidovudine
15 Ketogenic Diet Adjunctive to High Dose Methotrexate Chemotherapy for Primary Central Nervous System Lymphoma Unknown status NCT02983942 Phase 1, Phase 2
16 High-Dose Methotrexate Plus Steroid Followed by Concurrent Whole Brain Chemoradiation With Temozolomide for Immunocompetent Patients With Primary Central Nervous System Lymphoma - a Phase II Study Unknown status NCT00455286 Phase 2 Methotrexate;Methylprednisolone;Temozolomide
17 MT-R Followed by Autologous Stem Cells Transplantation in Newly-diagnosed Primary Central Nervous System Lymphoma Unknown status NCT02399189 Phase 2 R-MT followed by auto-HSCT
18 Immunochemotherapy in Primary Central Nervous System Lymphoma With Rituximab, HD-MTX, HD-Ara C, Cyclophosphamide, Ifosfamide, Vincristine, Vindesine, Temozolomide and DepoCyte Induction Followed by Maintenance Treatment in Elderly Patients With Temozolomide. Unknown status NCT01458730 Phase 2 Immunochemotherapy
19 Monoinstitutional Phase II Trial Addressing Tolerability and Activity of RCHOP Chemoimmunotherapy Preceded by BBB Permeabilization by t-NGR Necrosis Factor in Patients With Relapsed/Refractory Primary Central Nervous System Lymphoma Unknown status NCT03536039 Phase 2 NGR-hTNF;Doxorubicin;Cyclophosphamide;Vincristine;Prednisone
20 Multicenter Randomized Phase II Study of Methotrexate (MTX) and Temozolomide Versus MTX, Procarbazine, Vincristine and Cytarabine for Primary CNS Lymphoma (PCNSL) in the Elderly Unknown status NCT00503594 Phase 2 Methotrexate and temozolomide;Methotrexate , procarbazine ,vincristine ,cytarabine
21 Phase 2 Study of Temsirolimus in Refractory and Recurrent Primary CNS Lymphoma Unknown status NCT00942747 Phase 2 temsirolimus
22 Treatment of Patients Over 65 Years With Primary CNS Lymphoma: High-dose Methotrexate Combined With Chemo-immunotherapy Followed by Maintenance Therapy Unknown status NCT00989352 Phase 2 Methotrexate;Lomustine;Procarbazine;Rituximab
23 Phase II Trial of Pemetrexed and Temozolomide in Treating Patients With Relapsed PCNSL Unknown status NCT01985451 Phase 2 Pemetrexe;Temozolomide
24 Phase Ⅱ Trial of Temozolomide Plus Concurrent Whole-Brain Radiation Followed by TNV Regimen as Adjuvant Therapy for Patients With Newly Diagnosed Primary Central Nervous System (CNS) Lymphoma (PCNSL) Unknown status NCT01735747 Phase 2 nedaplatin;vincristine;Temozolomide
25 A Phase I Evaluation of the Combination of Pegylated Liposomal Doxorubicin (Doxil®) With PS-341 in Patients With Refractory Hematologic and Solid Malignancies Completed NCT00237627 Phase 1, Phase 2 PS-341;Doxil;Velcade
26 Ex Vivo Expanded Megakaryocytes for Supportive Care of Breast Cancer Patients: A Phase I/II Study Completed NCT00006225 Phase 1, Phase 2
27 Combination Chemotherapy (Methotrexate, Cyclophosphamide, And Etoposide Phosphate) Delivered In Conjunction With Osmotic Blood-Brain Barrier Disruption (BBBD), With Intraventricular Cytarabine +/- Intra-Ocular Chemotherapy, In Patients With Primary Central Nervous System Lymphoma Completed NCT00074178 Phase 2 cyclophosphamide;cytarabine;dexamethasone;etoposide phosphate;methotrexate
28 Combination Chemotherapy (Methotrexate, Procarbazine And CCNU), Intraventricular Cytarabine And Methotrexate, +/- Intra-Ocular Chemotherapy For Patients With Primary Central Nervous System Lymphoma Completed NCT00074191 Phase 2 cytarabine;dexamethasone;lomustine;methotrexate;procarbazine hydrochloride
29 A Phase II Trial Of Buparlisib (BKM120) In Patients With Recurrent/Refractory Primary Central Nervous System Lymphoma (PCNSL) and Recurrent/Refractory Secondary Central Nervous System Lymphoma (SCNSL) Completed NCT02301364 Phase 2 Buparlisib (BKM120)
30 Phase II Study Evaluating the Efficacy of Lenalidomide in Association With Rituximab in Refractory or Relapse of Primary Central Nervous System Lymphoma (PCNSL) Completed NCT01956695 Phase 2 Lenalidomide;Rituximab
31 AIDS-Related Primary Central Nervous System Lymphoma: A Phase II Pilot Study of High-Dose Intravenous Methotrexate With Rituximab Leucovorin Rescue and Highly Active Antiretroviral Therapy Completed NCT00267865 Phase 2 Methotrexate;Rituximab;Leucovorin
32 A Phase 2 Study of Idarubicin Based Combined Modality Therapy in Primary Central Nervous System Lymphoma Completed NCT00193973 Phase 2 Idarubicin, Methotrexate, Filgrastim, intrathecal Ara-C
33 Open-label, Non-randomized, Phase 2 Study Evaluating Efficacy and Safety of PQR309 in Patients With Relapsed or Refractory Primary Central Nervous System Lymphoma Completed NCT02669511 Phase 2 PQR309
34 A Phase II Trial Of Induction Therapy With Zidovudine, Interleukin-2, And Ganciclovir In The Treatment Of HIV Positive Primary Central Nervous System Lymphoma Completed NCT00006264 Phase 2 ganciclovir;zidovudine
35 Randomized Phase II Trial on Primary Chemotherapy With High-dose Methotrexate, Alone or Associated With High-dose Cytarabine, Followed by Response- and Age-tailored Radiotherapy for Immunocompetent Patients With Newly Diagnosed Primary Central Nervous System Lymphoma Completed NCT00210314 Phase 2 high dose methotrexate;high dose cytarabine
36 Phase I/II Study Of Pre-Irradiation Chemotherapy With Methotrexate, Rituximab, And Temozolomide And Post -Irradiation Temozolomide For Primary Central Nervous System Lymphoma Completed NCT00068250 Phase 1, Phase 2 rituximab;methotrexate;temozolomide 100 mg/m^2;temozolomide 150 mg/m^2;temozolomide 200 mg/m^2;post-radiation therapy temozolomide
37 A Phase II Study of Intensive Methotrexate and Cytarabine Followed by High Dose Beam Chemotherapy With Autologous Peripheral Blood Progenitor Cell Transplantation in Patients With Newly Diagnosed Primary Central Nervous System Lymphoma Completed NCT00003632 Phase 2 carmustine;cytarabine;etoposide;melphalan;methotrexate
38 Evaluating the MBVP Chemotherapy Schedule Followed by Consolidating Radiotherapy in Non-AIDS Related Primary Central Nervous System Lymphoma (NAPCL) Completed NCT00003061 Phase 2 cytarabine;methotrexate
39 Phase II Trial of Sequential Chemotherapy and Radiotherapy for AIDS-Related Primary Central Nervous System Lymphoma Completed NCT00000801 Phase 2 Filgrastim;Vincristine sulfate;Doxorubicin hydrochloride;Cyclophosphamide;Cytarabine;Dexamethasone
40 Phase II Trial of Hihg-Dose Thiotepa, Busulfan, Cyclophosphamide, and Rituximab With Autologous Stem Cell Transplantation for Patients With CNS Involvement by Non-Hodgkin's Lymphoma or Primary CNS Lymphoma Completed NCT01182415 Phase 2 High-dose chemotherapy
41 Intensive Chemotherapy And Immunotherapy In Patients With Newly Diagnosed Primary CNS Lymphoma Completed NCT00098774 Phase 2 cytarabine;etoposide;leucovorin calcium;methotrexate;temozolomide
42 Hydroxyurea Treatment of EBV-Associated Primary CNS Lymphoma in Children and Adults With AIDS Completed NCT00002940 Phase 2 hydroxyurea
43 Open-labeled, Multicenter Phase II Study of High-Dose (HD) Methotrexate (MTX) Induction Chemotherapy Followed by Alternative HD MTX-based and HD Cytarabine-based Combination Consolidation Chemotherapy for Newly Diagnosed Primary CNS Lymphoma; CISL 10-01 Study Completed NCT01083342 Phase 2 MTX, MVD, VIA
44 A Phase II Study of Methotrexate and Thiotepa Chemotherapy for Patients With Newly Diagnosed Primary CNS Lymphoma Completed NCT00045539 Phase 2 leucovorin calcium;methotrexate;thiotepa
45 Randomized Phase II Trial On Primary Chemotherapy With High-Dose Methotrexate And High-Dose Cytarabine With Or Without Thiotepa, And With Or Without Rituximab, Followed By Brain Irradiation Vs. High-Dose Chemotherapy Supported By Autologous Stem Cells Transplantation For Immunocompetent Patients With Newly Diagnosed Primary CNS Lymphoma Completed NCT01011920 Phase 2 Methotrexate;Ara-C;Rituximab;Thiotepa;BCNU
46 Primary Central Nervous System Non-Hodgkins's Lymphoma (PCNSL): A Phase II Clinical Trial of Radiation Therapy and High Dose Corticosteroids for Elderly Patients (70 Years of Age and Older) Completed NCT00003278 Phase 2 dexamethasone
47 PHASE II TRIAL OF CHEMOTHERAPY PLUS RADIOTHERAPY FOR MANAGEMENT OF PRIMARY CENTRAL NERVOUS SYSTEM NON-HODGKIN'S LYMPHOMA (PCNSL) Completed NCT00002676 Phase 2 carmustine;cyclophosphamide;cytarabine;dexamethasone;doxorubicin hydrochloride;methotrexate;vincristine sulfate
48 Phase I Trial of Escalating High Dose Methotrexate Supported by Glucarpidase to Treat Patients With Primary Central Nervous Lymphoma (PCNSL) Completed NCT00727831 Phase 1, Phase 2 glucarpidase;leucovorin calcium;methotrexate
49 Phase IB/II Study Combining the PI3K Inhibitor Copanlisib With the BTK Inhibitor Ibrutinib in Patients With Recurrent/Refractory Primary CNS Lymphoma Recruiting NCT03581942 Phase 1, Phase 2 Ibrutinib;Copanlisib
50 Randomized Phase II Trial on Fitness- and Comorbidity- Tailored Treatment in Elderly Patients With Newly Diagnosed Primary CNS Lymphoma (FIORELLA Trial) Recruiting NCT03495960 Phase 2 Rituximab;Methotrexate;Procarbazine;Lenalidomide;Temozolomide

Search NIH Clinical Center for Primary Central Nervous System Lymphoma

Genetic Tests for Primary Central Nervous System Lymphoma

Anatomical Context for Primary Central Nervous System Lymphoma

MalaCards organs/tissues related to Primary Central Nervous System Lymphoma:

40
Spinal Cord, Eye, Brain, Liver, T Cells, Pituitary, Bone Marrow

Publications for Primary Central Nervous System Lymphoma

Articles related to Primary Central Nervous System Lymphoma:

(show top 50) (show all 2515)
# Title Authors PMID Year
1
Primary CNS lymphoma in the elderly: temozolomide therapy and MGMT status. 61 54
19841864 2010
2
Del(6)(q22) and BCL6 rearrangements in primary CNS lymphoma are indicators of an aggressive clinical course. 61 54
18645192 2008
3
Chromosomal translocations fusing the BCL6 gene to different partner loci are recurrent in primary central nervous system lymphoma and may be associated with aberrant somatic hypermutation or defective class switch recombination. 61 54
16896311 2006
4
Gene expression and angiotropism in primary CNS lymphoma. 54 61
16418334 2006
5
Expression of the chemokine receptors CXCR4, CXCR5, and CCR7 in primary central nervous system lymphoma. 54 61
15967804 2005
6
BCL-6 expression predicts improved survival in patients with primary central nervous system lymphoma. 54 61
12631608 2003
7
Expression of B-cell-attracting chemokine 1 (CXCL13) by malignant lymphocytes and vascular endothelium in primary central nervous system lymphoma. 61 54
12393412 2003
8
Primary central nervous system lymphomas express Vh genes with intermediate to high somatic mutations. 54 61
11378551 2001
9
AIDS primary central nervous system lymphoma: molecular analysis of the expressed VH genes and possible implications for lymphomagenesis. 61 54
9973412 1999
10
Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review. 61
32253501 2021
11
Primary central nervous system lymphoma presenting with Parkinsonism as an initial manifestation: A case report and literature review. 61
33767864 2021
12
Dynamic contrast-enhanced magnetic resonance imaging biomarkers predict chemotherapeutic responses and survival in primary central-nervous-system lymphoma. 61
32997181 2021
13
MMSE is an independent prognostic factor for survival in primary central nervous system lymphoma. 61
33611761 2021
14
Primary Central Nervous System Lymphoma Mimicking Longitudinally Extensive Transverse Myelitis. 61
33791064 2021
15
Case report of primary dural lymphoma mimicking a cerebellar meningioma and brief review of literature. 61
31301042 2021
16
Glioblastoma and primary central nervous system lymphoma: differentiation using MRI derived first-order texture analysis - a machine learning study. 61
33657924 2021
17
A Rapid Genotyping Panel for Detection of Primary Central Nervous System Lymphoma. 61
33735913 2021
18
Movement disorders in primary central nervous system lymphoma: two unreported cases and a review of literature. 61
33443666 2021
19
Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (Review). 61
33650642 2021
20
EBV-associated primary CNS lymphoma occurring after immunosuppression is a distinct immunobiological entity. 61
33202420 2021
21
Emerging insights into origin and pathobiology of primary central nervous system lymphoma. 61
33766752 2021
22
Primary CNS lymphoma after CLIPPERS: a case series. 61
33789924 2021
23
Primary Central Nervous System Lymphoma - Ocular Variant (PCNSL-O): An Interdisciplinary Review on Management. 61
33762182 2021
24
Sorting biologic subtypes of primary CNS lymphoma. 61
33734333 2021
25
Primary central nervous system lymphoma mimicking autoimmune encephalitis: the role of autoantibody testing. 61
32990859 2021
26
Absence of meaningful neurocognitive recovery in comatose patients with primary central nervous system lymphoma despite an effective response to chemotherapy: Case reports. 61
33575028 2021
27
The process of somatic hypermutation increases polyreactivity for central nervous system antigens in primary central nervous system lymphoma. 61
32193251 2021
28
Diagnostic Yield of Body CT and Whole-Body FDG PET/CT for Initial Systemic Staging in Patients With Suspected Primary CNS Lymphoma: A Systematic Review and Meta-Analysis. 61
32812800 2021
29
Deep Learning for Automatic Differential Diagnosis of Primary Central Nervous System Lymphoma and Glioblastoma: Multi-Parametric Magnetic Resonance Imaging Based Convolutional Neural Network Model. 61
33694250 2021
30
Validation of combined use of DWI and percentage signal recovery-optimized protocol of DSC-MRI in differentiation of high-grade glioma, metastasis, and lymphoma. 61
32821962 2021
31
[Tumefactive demyelinating lesion (TDL)]. 61
33762460 2021
32
Juxtacortical susceptibility changes in progressive multifocal leukoencephalopathy at the gray-white matter junction correlates with iron-enriched macrophages. 61
33749379 2021
33
High-dose methotrexate monotherapy for newly diagnosed primary central nervous system lymphoma: 15-year multicenter experience. 61
32978898 2021
34
Primary Central Nervous System Lymphoma (PCNSL) - A Rare and Unusual Presentation of Rapidly Progressive Major Cognitive Dysfunction. 61
33527818 2021
35
Primary therapy and survival in patients aged over 70-years-old with primary central nervous system lymphoma: a contemporary, nationwide, population-based study in the Netherlands. 61
32241841 2021
36
Gamma knife stereotactic radiosurgery as an effective tool in primary CNS lymphoma: Evaluation of stereotactic radiosurgery and methotrexate treatment in a prospective and observational clinical research study. 61
33418336 2021
37
What Is on Your Mind? Impaired Social Cognition in Primary Central Nervous System Lymphoma Patients Despite Ongoing Complete Remission. 61
33668180 2021
38
Neurocognitive functioning and radiologic changes in primary CNS lymphoma patients: results from the HOVON 105/ ALLG NHL 24 randomised controlled trial. 61
33560442 2021
39
Evaluation of the Temporal Muscle Thickness as an Independent Prognostic Biomarker in Patients with Primary Central Nervous System Lymphoma. 61
33540564 2021
40
Changes in cerebrospinal fluid interleukin-10 levels display better performance in predicting disease relapse than conventional magnetic resonance imaging in primary central nervous system lymphoma. 61
33618687 2021
41
Resection of primary central nervous system lymphoma: impact of patient selection on overall survival. 61
33636699 2021
42
Primary Central Nervous System Lymphoma: Evolving Biologic Insights and Recent Therapeutic Advances. 61
33288483 2021
43
Uveitis intermedia as the initial presentation of a primary lymphoma of the central nervous system. 61
33640210 2021
44
CSF interleukin 6 is a useful marker to distinguish pseudotumoral CNS inflammatory diseases from primary CNS lymphoma. 61
33609156 2021
45
MYD88 L265P mutation and interleukin-10 detection in cerebrospinal fluid are highly specific discriminating markers in patients with primary central nervous system lymphoma: results from a prospective study. 61
33620087 2021
46
Differentiating Glioblastoma from Primary Central Nervous System Lymphoma: The Value of Shaping and Nonenhancing Peritumoral Hyperintense Gyral Lesion on FLAIR Imaging. 61
33548537 2021
47
Consensus Recommendations for MRI and PET Imaging of Primary Central Nervous System Lymphoma: Guideline Statement from the International Primary CNS Lymphoma Collaborative Group (IPCG). 61
33560416 2021
48
New genetic prognostic biomarkers in primary central nervous system lymphoma (PCNSL). 61
33591648 2021
49
Inflammatory Brain Lesions as Omen of Primary Central Nervous System Lymphoma: A Case Report and Literature Review. 61
33557224 2021
50
Seizures in steroid-responsive encephalopathy. 61
33219869 2021

Variations for Primary Central Nervous System Lymphoma

Copy number variations for Primary Central Nervous System Lymphoma from CNVD:

7 (show top 50) (show all 54)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 13396 1 1 2300000 Gain Primary central nervous system lymphoma
2 40586 10 130500000 135374737 Gain Primary central nervous system lymphoma
3 48335 11 1 2800000 Gain Primary central nervous system lymphoma
4 50189 11 115400000 120700000 Gain Primary central nervous system lymphoma
5 57101 11 63100000 67100000 Gain Primary central nervous system lymphoma
6 81492 14 103000000 106368585 Gain Primary central nervous system lymphoma
7 81496 14 103000000 106368585 Loss Primary central nervous system lymphoma
8 94763 15 70400000 73100000 Gain Primary central nervous system lymphoma
9 97352 16 1 6300000 Gain Primary central nervous system lymphoma
10 99609 16 27600000 34400000 Gain Primary central nervous system lymphoma
11 106267 16 87200000 88827254 Gain Primary central nervous system lymphoma
12 106863 17 1 3600000 Gain P2RX5 Primary central nervous system lymphoma
13 106864 17 1 3600000 Gain EMC6 Primary central nervous system lymphoma
14 117583 17 72900000 78774742 Gain Primary central nervous system lymphoma
15 124164 19 1 6900000 Gain Primary central nervous system lymphoma
16 128872 19 43400000 47800000 Gain Primary central nervous system lymphoma
17 130166 19 47800000 57600000 Gain Primary central nervous system lymphoma
18 132108 19 57600000 63811651 Gain Primary central nervous system lymphoma
19 155738 20 57900000 62435964 Gain Primary central nervous system lymphoma
20 159204 21 41400000 46944323 Gain Primary central nervous system lymphoma
21 162471 22 21800000 24300000 Loss Primary central nervous system lymphoma
22 165691 22 48200000 49691432 Gain IL17REL Primary central nervous system lymphoma
23 165692 22 48200000 49691432 Gain MLC1 Primary central nervous system lymphoma
24 168173 3 127700000 131500000 Gain Primary central nervous system lymphoma
25 168662 3 131500000 135700000 Gain Primary central nervous system lymphoma
26 168981 3 1357001 1525502 Deletion TPO Primary central nervous system lymphoma
27 173428 3 193800000 199501827 Gain Primary central nervous system lymphoma
28 180220 4 101538006 101658202 Deletion EMCN Primary central nervous system lymphoma
29 189173 4 71126403 71135557 Deletion FDCSP Primary central nervous system lymphoma
30 189355 4 73116384 73237614 Deletion NPFFR2 Primary central nervous system lymphoma
31 199429 5 38881884 38970159 Deletion OSMR Primary central nervous system lymphoma
32 203980 6 104800000 113900000 Loss Primary central nervous system lymphoma
33 204682 6 113900000 118600000 Loss Primary central nervous system lymphoma
34 205017 6 118600000 130400000 Loss Primary central nervous system lymphoma
35 207626 6 159510416 159613130 Deletion FNDC1 Primary central nervous system lymphoma
36 207757 6 160900000 164400000 Loss Primary central nervous system lymphoma
37 211018 6 31900000 33600000 Loss Primary central nervous system lymphoma
38 215228 6 73300000 139100000 Loss Primary central nervous system lymphoma
39 215399 6 75850761 75972343 Deletion COL12A1 Primary central nervous system lymphoma
40 215419 6 75900000 87500000 Loss Primary central nervous system lymphoma
41 215890 6 82700000 145700000 Loss Primary central nervous system lymphoma
42 216158 6 87500000 92100000 Loss Primary central nervous system lymphoma
43 216419 6 92100000 104800000 Loss Primary central nervous system lymphoma
44 217057 7 1 2100000 Gain Primary central nervous system lymphoma
45 221886 7 147500000 152200000 Gain Primary central nervous system lymphoma
46 227727 7 71800000 77400000 Gain Primary central nervous system lymphoma
47 230089 7 97900000 104400000 Gain Primary central nervous system lymphoma
48 233710 8 12700000 19100000 Loss Primary central nervous system lymphoma
49 234942 8 140000000 146274826 Gain Primary central nervous system lymphoma
50 236937 8 19100000 23400000 Loss Primary central nervous system lymphoma

Expression for Primary Central Nervous System Lymphoma

Search GEO for disease gene expression data for Primary Central Nervous System Lymphoma.

Pathways for Primary Central Nervous System Lymphoma

Pathways related to Primary Central Nervous System Lymphoma according to KEGG:

36
# Name Kegg Source Accession
1 B cell receptor signaling pathway hsa04662

GO Terms for Primary Central Nervous System Lymphoma

Biological processes related to Primary Central Nervous System Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 defense response to bacterium GO:0042742 9.5 MYD88 IGHM CXCL13
2 humoral immune response GO:0006959 9.4 PAX5 BCL2
3 positive regulation of B cell proliferation GO:0030890 9.37 BCL6 BCL2
4 regulation of transmembrane transporter activity GO:0022898 9.26 PIM1 BCL2
5 cytokine-mediated signaling pathway GO:0019221 9.26 PIM1 MYD88 BCL6 BCL2
6 germinal center formation GO:0002467 9.16 CXCL13 BCL6
7 negative regulation of apoptotic process GO:0043066 9.02 PIM1 MYD88 MGMT BCL6 BCL2

Sources for Primary Central Nervous System Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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