PCNSL
MCID: PRM226
MIFTS: 48

Primary Central Nervous System Lymphoma (PCNSL)

Categories: Blood diseases, Cancer 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 52 58 36 54 71
Primary Brain Lymphoma 52 58
Primary Cns Lymphoma 52 58
Pcnsl 52 58
Primary Cerebral Lymphoma 71
Primary Lymphoma, Cns 52

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 72 C0240803 C0280803
Orphanet 58 ORPHA46135
UMLS 71 C0240803 C0280803

Summaries for Primary Central Nervous System Lymphoma

NIH Rare Diseases : 52 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 Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, b cells and eye, 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 : 74 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 362)
# Related Disease Score Top Affiliating Genes
1 intraocular lymphoma 32.0 PAX5 CXCR5 BCL6 BCL2
2 central nervous system lymphoma 31.5 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
3 diffuse large b-cell lymphoma 31.1 PAX5 MYD88 BCL6
4 lymphoma, hodgkin, classic 31.1 PAX5 BCL6 BCL2
5 lymphosarcoma 30.7 BCL6 BCL2
6 human herpesvirus 8 30.4 BCL6 BCL2
7 lymphoma, non-hodgkin, familial 30.3 PAX5 MYD88 CXCR5 BCL6 BCL2
8 primary mediastinal large b-cell lymphoma 30.3 BCL6 BCL2
9 intravascular large b-cell lymphoma 30.2 MYD88 BCL6 BCL2
10 marginal zone b-cell lymphoma 30.0 PAX5 MYD88 BCL6
11 peripheral t-cell lymphoma 29.9 PAX5 CXCL13 BCL6
12 lymphoma 29.9 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
13 lymphoplasmacytic lymphoma 29.8 PAX5 MYD88
14 reticulosarcoma 29.7 PAX5 BCL6 BCL2
15 leukemia, acute lymphoblastic 3 29.5 PAX5 CXCR5 CXCL13
16 b-cell lymphoma 29.5 PAX5 MYD88 MGMT CXCR5 CXCL13 BCL6
17 angioimmunoblastic t-cell lymphoma 29.3 CXCL13 BCL6
18 follicular lymphoma 28.9 PAX5 IGHM CXCR5 BCL6 BCL2
19 burkitt lymphoma 28.6 PAX5 IGHM CXCR5 CXCL13 BCL6 BCL2
20 leukemia, chronic lymphocytic 28.6 PAX5 MYD88 IGHM CXCR5 BCL6 BCL2
21 lymphoma, mucosa-associated lymphoid type 28.4 PAX5 MYD88 CXCR5 CXCL13 BCL6 BCL2
22 acquired immunodeficiency syndrome 10.8
23 glioblastoma multiforme 10.8
24 glioma 10.6
25 glial tumor 10.6
26 rare tumor 10.6
27 human immunodeficiency virus type 1 10.5
28 toxoplasmosis 10.5
29 neutropenia 10.5
30 immune deficiency disease 10.4
31 uveitis 10.4
32 ataxia and polyneuropathy, adult-onset 10.4
33 hydrocephalus 10.4
34 lymphoproliferative syndrome 10.4
35 intracranial hypertension 10.4
36 systemic lupus erythematosus 10.3
37 demyelinating disease 10.3
38 cerebral lymphoma 10.3
39 progressive multifocal leukoencephalopathy 10.3
40 meningitis 10.3
41 encephalitis 10.3
42 marek disease 10.3
43 multiple sclerosis 10.2
44 pars planitis 10.2
45 anaplastic large cell lymphoma 10.2
46 mucositis 10.2
47 astrocytoma 10.2
48 brain edema 10.2
49 diabetes insipidus 10.2
50 encephalopathy 10.2

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:

45
# 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 222)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Teniposide Approved Phase 4 29767-20-2 34698
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
3
rituximab Approved Phase 4 174722-31-7 10201696
4
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
5
Cytarabine Approved, Experimental, Investigational Phase 4 147-94-4, 65-46-3 6253
6
Vincristine Approved, Investigational Phase 4 57-22-7, 2068-78-2 5978
7
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
8
Etoposide Approved Phase 4 33419-42-0 36462
9
Ifosfamide Approved Phase 4 3778-73-2 3690
10
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
11
Cisplatin Approved Phase 4 15663-27-1 2767 441203 84093
12
Gemcitabine Approved Phase 4 95058-81-4 60750
13
Epirubicin Approved Phase 4 56420-45-2 41867
14
Oxaliplatin Approved, Investigational Phase 4 61825-94-3 43805 6857599 5310940 9887054
15
Fotemustine Investigational Phase 4 92118-27-9
16 Formoterol Fumarate Phase 4
17 Etoposide phosphate Phase 4
18
Isophosphamide mustard Phase 4 0
19 Immunoglobulins Phase 4
20 Antibodies Phase 4
21 Antibodies, Monoclonal Phase 4
22
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
23
Cefepime Approved, Investigational Phase 3 88040-23-7 5479537
24
Levofloxacin Approved, Investigational Phase 3 100986-85-4 149096
25
Ofloxacin Approved Phase 3 82419-36-1 4583
26
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
27
Clotrimazole Approved, Vet_approved Phase 3 23593-75-1 2812
28
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
29
Ondansetron Approved Phase 3 99614-02-5 4595
30
Dalteparin Approved Phase 3 9005-49-6
31
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
32
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
33
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 2826718 468682
34
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
35
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
36
Idarubicin Approved Phase 2, Phase 3 58957-92-9 42890
37
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
38 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
39
protease inhibitors Phase 3
40 HIV Protease Inhibitors Phase 3
41 BB 1101 Phase 3
42 Cytochrome P-450 Enzyme Inhibitors Phase 3
43 Anesthetics, Dissociative Phase 3
44 Dermatologic Agents Phase 3
45 Anti-Bacterial Agents Phase 3
46 Gastrointestinal Agents Phase 3
47 Hormones Phase 3
48 Antiemetics Phase 3
49 Antifungal Agents Phase 3
50 Analgesics Phase 3

Interventional clinical trials:

(show top 50) (show all 176)
# 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 Study of PD-1 Monoclonal Antibody in Combination With Chemotherapy in Patients With Relapsed or Refractory Non-Hodgkin Lymphoma Not yet recruiting NCT04134247 Phase 4 PD-1 combined with chemotherapy;chemotherapy
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 Multicenter, Double-Blind, Randomized Study to Compare the Safety and Efficacy of Levofloxacin With That of Cefepime in the Treatment of Fever and Neutropenia - Phase IIIB Unknown status NCT00020865 Phase 3 cefepime hydrochloride;levofloxacin
7 A Randomized Double-Blind Controlled Trial of Ketamine Versus Placebo in Conjunction With Best Pain Management in Neuropathic Pain in Cancer Patients Unknown status NCT01316744 Phase 3 ketamine hydrochloride
8 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
9 Phase III, Randomized, Double-Blind, Placebo-Controlled Crossover Trial of Ondansetron in the Control of Chronic Nausea and Vomiting Not Due to Antineoplastic Therapy in Patients With Advanced Cancer Completed NCT00006348 Phase 3 ondansetron
10 A Phase III Double-Blind Equivalence Study of Two Different Formulations of Slow-Release Morphine Followed by a Randomization Between Dextromethorphan or Placebo Plus Statex SR for Chronic Cancer Pain Relief in Terminally Ill Patients Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
11 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
12 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
13 A Multicenter, Double-Blind, Randomized, Comparative Study To Evaluate The Safety, Tolerability, And Efficacy Of MK-0991 Versus (Amphotericin B) Liposome For Injection As Empirical Therapy In Patients With Persistent Fever And Neutropenia Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
14 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
15 Treatment of Primary CNS Lymphoma With Systemic R-IDARAM Chemotherapy and Intrathecal Immunochemotherapy Recruiting NCT02657785 Phase 2, Phase 3 R-IDARAM plus intrathecal chemotherapy
16 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
17 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)
18 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
19 Temozolomide in Treatment of Patients With Primary Central Nervous System Lymphoma Unknown status NCT02934204 Phase 2 methotrexate;temozolomide
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 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
22 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
23 Ketogenic Diet Adjunctive to High Dose Methotrexate Chemotherapy for Primary Central Nervous System Lymphoma Unknown status NCT02983942 Phase 1, Phase 2
24 Open-label Single Arm Phase II Study on Pembrolizumab for Recurrent Primary Central Nervous System Lymphoma (PCNSL) Unknown status NCT02779101 Phase 2 pembrolizumab
25 Phase 2 Study of Temsirolimus in Refractory and Recurrent Primary CNS Lymphoma Unknown status NCT00942747 Phase 2 temsirolimus
26 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
27 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
28 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
29 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
30 Phase II Trial of Pemetrexed and Temozolomide in Treating Patients With Relapsed PCNSL Unknown status NCT01985451 Phase 2 Pemetrexe;Temozolomide
31 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
32 Pilot Study Of PMitCEBO Plus G-CSF In Good-Prognosis HIV-Related Lymphoma Unknown status NCT00032149 Phase 1, Phase 2 cyclophosphamide;etoposide;mitoxantrone hydrochloride;prednisolone;vincristine sulfate
33 Freiburg ZNS-NHL Study: Therapy for Patients With Primary Non-Hodgkin Lymphoma of the CNS - Sequential High Dosage Chemotherapy With Autologous Peripheral Blood Stem Cell Plantation Unknown status NCT00647049 Phase 2 methotrexate;Rituximab;Cytarabine;Thiotepa;Carmustin
34 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
35 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
36 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
37 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
38 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)
39 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
40 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
41 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
42 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
43 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
44 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
45 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
46 Hydroxyurea Treatment of EBV-Associated Primary CNS Lymphoma in Children and Adults With AIDS Completed NCT00002940 Phase 2 hydroxyurea
47 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
48 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
49 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
50 Pilot Study in AIDS-Related Lymphomas Completed NCT00002524 Phase 2 Cisplatin;Cyclophosphamide;Cytarabine;Doxorubicin Hydrochloride (DOX);Etoposide;Fluorouracil;Ifosfamide;Leucovorin calcium;Methotrexate;Methylprednisolone;Pentamidine;Prednisone;Trimethoprim-Sulfamethoxazole;Vincristine Sulfate;Zidovudine (AZT)

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
Brain, B Cells, Eye, T Cells, Spinal Cord, Liver, Bone

Publications for Primary Central Nervous System Lymphoma

Articles related to Primary Central Nervous System Lymphoma:

(show top 50) (show all 2364)
# Title Authors PMID Year
1
Primary CNS lymphoma in the elderly: temozolomide therapy and MGMT status. 54 61
19841864 2010
2
Del(6)(q22) and BCL6 rearrangements in primary CNS lymphoma are indicators of an aggressive clinical course. 54 61
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. 54 61
16896311 2006
4
Gene expression and angiotropism in primary CNS lymphoma. 61 54
16418334 2006
5
Expression of the chemokine receptors CXCR4, CXCR5, and CCR7 in primary central nervous system lymphoma. 61 54
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. 54 61
12393412 2003
8
Primary central nervous system lymphomas express Vh genes with intermediate to high somatic mutations. 61 54
11378551 2001
9
AIDS primary central nervous system lymphoma: molecular analysis of the expressed VH genes and possible implications for lymphomagenesis. 54 61
9973412 1999
10
Eye involvement in primary central nervous system lymphoma. 61
32057762 2020
11
The Challenges of Applying Radiation in Primary Central Nervous System Lymphoma. 61
32531380 2020
12
Durable Survival Outcomes in Primary and Secondary Central Nervous System Lymphoma After High-dose Chemotherapy and Autologous Stem Cell Transplantation Using a Thiotepa, Busulfan, and Cyclophosphamide Conditioning Regimen. 61
32229199 2020
13
Effectiveness of digital PCR for MYD88L265P detection in vitreous fluid for primary central nervous system lymphoma diagnosis. 61
32536998 2020
14
Conventional MR and DW imaging findings of cerebellar primary CNS lymphoma: comparison with high-grade glioma. 61
32561819 2020
15
High incidence of venous thromboembolism and major bleeding in patients with primary CNS lymphoma. 61
32573292 2020
16
Primary Central Nervous System Lymphoma With Diffuse Neurolymphomatosis Involving Multiple Cranial and Spinal Nerve Roots. 61
32332304 2020
17
Correction to: atypical radiological findings of primary central nervous system lymphoma. 61
32211926 2020
18
Pemetrexed in Recurrent or Progressive Central Nervous System Lymphoma: A Phase I Multicenter Clinical Trial. 61
32520407 2020
19
Diagnostic delay and outcome in immunocompetent patients with primary central nervous system lymphoma in Spain: a multicentric study. 61
32524392 2020
20
Expression of hypoxia-inducible factor 1α, glucose transporter 1, and hexokinase 2 in primary central nervous system lymphoma and the correlation with the biological behaviors. 61
32533646 2020
21
Visual hallucinations as psychiatric onset of a primary central nervous system lymphoma: a case report and a brief review of literature on neoplasm-correlated neuropsychiatric disorders. 61
32524322 2020
22
Metabolome Analysis Reveals Excessive Glycolysis via PI3K/AKT/mTOR and RAS/MAPK Signaling in Methotrexate-Resistant Primary CNS Lymphoma-Derived Cells. 61
32108030 2020
23
High-dose methotrexate in ICU patients: a retrospective study. 61
32535662 2020
24
Risk Factors for Early Mortality in Patients with Primary Central Nervous System Lymphoma: A Large-Cohort Retrospective Study. 61
32251807 2020
25
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment. 61
32452898 2020
26
Primary dural lymphomas: Clinical presentation, management, and outcome. 61
32176324 2020
27
Tirabrutinib: First Approval. 61
32382949 2020
28
Sex-Specific Differences in Primary CNS Lymphoma. 61
32560244 2020
29
On Point in Primary CNS Lymphoma. 61
32510610 2020
30
A diagnostic challenge of primary Central nervous system lymphoma: from the eyes to the brain. 61
32449877 2020
31
Atypical radiological findings of primary central nervous system lymphoma. 61
32077984 2020
32
Consecutive single-institution case series of primary central nervous system lymphoma treated by R-MPV or high-dose methotrexate monotherapy. 61
32469065 2020
33
Usefulness of carbon-11-labeled methionine positron-emission tomography for assessing the treatment response of primary central nervous system lymphoma. 61
32129443 2020
34
Use of FDG-PET/CT for systemic assessment of suspected primary central nervous system lymphoma: a LOC study. 61
32405997 2020
35
Advances and challenges in the treatment of primary central nervous system lymphoma. 61
32420657 2020
36
Cerebrospinal Fluid Flow Cytometry: Utility in Central Nervous System Lymphoma Diagnosis. 61
32228724 2020
37
Letter to the Editor Regarding Effects of the COVID-19 Outbreak in Northern Italy: Perspectives from the Bergamo Neurosurgery Department, and the Role of Radiosurgery as a Minimally Invasive Procedure for Primary Central Nervous System Lymphoma in the Pandemic Outbreak. 61
32380338 2020
38
Maculopathy Associated With Osmotic Blood- Brain Barrier Disruption and Chemotherapy in Patients With Primary CNS Lymphoma. 61
32484895 2020
39
Current status and quality of radiomics studies in lymphoma: a systematic review. 61
32472274 2020
40
Apparent diffusion coefficient as a valuable quantitative parameter for predicting clinical outcomes in patients with newly diagnosed primary CNS lymphoma. 61
32408414 2020
41
GSEA-assisted gene signatures valid for combinations of prognostic markers in PCNSL. 61
32439996 2020
42
Cost-effectiveness analysis of rituximab with methotrexate, cytarabine and thiotepa for the treatment of patients with primary central nervous system lymphoma. 61
31931647 2020
43
Health-related quality of life after chemotherapy with or without rituximab in primary central nervous system lymphoma patients: results from a randomised phase III study. 61
32371123 2020
44
Clinicopathological characteristics of primary central nervous system lymphoma with low 18F-fludeoxyglucose uptake on brain positron emission tomography. 61
32443328 2020
45
Targeting the PI3K/AKT/mTOR Signaling Pathway in Primary Central Nervous System Lymphoma: Current Status and Future Prospects. 61
32416683 2020
46
Reduced frequency of Intravitreal methotrexate injection lowers the risk of Keratopathy in Vitreoretinal lymphoma patients. 61
32397978 2020
47
LCK expression is a potential biomarker for distinguishing primary central nervous system lymphoma from glioblastoma multiforme. 61
32237064 2020
48
Cell-Block cytology in diagnosis of primary central nervous system lymphoma: A case report. 61
32243381 2020
49
Clinical Characteristics, Surgical Outcomes, and Prognostic Factors of Intracranial Primary Central Nervous System Lymphoma. 61
32311566 2020
50
Allogeneic hematopoietic stem cell transplantation for primary central nervous system lymphoma. 61
31399528 2020

Variations for Primary Central Nervous System Lymphoma

Cosmic variations for Primary Central Nervous System Lymphoma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM105231581 MYD88 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.818T>C p.L273P 3:38141150-38141150 0

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 TMEM93 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 C4orf7 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

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

# Super pathways Score Top Affiliating Genes
1 11.54 PIM1 PAX5 MYD88 CXCR5 BCL6
2 11.39 PIM1 BCL6 BCL2
3 11.03 PIM1 BCL2
4 11.02 PIM1 PAX5 BCL2
5
Show member pathways
10.9 MGMT BCL2

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 9.54 MYD88 IGHM CXCL13
2 cytokine-mediated signaling pathway GO:0019221 9.46 PIM1 MYD88 BCL6 BCL2
3 humoral immune response GO:0006959 9.43 PAX5 BCL2
4 positive regulation of B cell proliferation GO:0030890 9.4 BCL6 BCL2
5 leukocyte chemotaxis GO:0030595 9.32 CXCR5 CXCL13
6 germinal center formation GO:0002467 9.26 CXCL13 BCL6
7 negative regulation of apoptotic process GO:0043066 9.02 PIM1 MYD88 MGMT BCL6 BCL2
8 regulation of transmembrane transporter activity GO:0022898 8.96 PIM1 BCL2

Sources for Primary Central Nervous System Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....