PCNSL
MCID: PRM226
MIFTS: 52

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 53 59 55 73
Primary Brain Lymphoma 53 59
Primary Cns Lymphoma 53 59
Pcnsl 53 59
Primary Cerebral Lymphoma 73
Primary Lymphoma, Cns 53

Characteristics:

Orphanet epidemiological data:

59
primary central nervous system lymphoma
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA46135
ICD10 via Orphanet 34 C83.3
UMLS via Orphanet 74 C0240803 C0280803

Summaries for Primary Central Nervous System Lymphoma

NIH Rare Diseases : 53 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 NF-kappaB Signaling and IL2 signaling events mediated by PI3K. The drugs leucovorin and Methotrexate 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

Wikipedia : 76 A primary central nervous system lymphoma (PCNSL), also known as microglioma and primary brain lymphoma,... 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 130)
# Related Disease Score Top Affiliating Genes
1 intraocular lymphoma 32.5 BCL6 PAX5
2 central nervous system lymphoma 32.3 BCL6 CD274 IL2 MGMT MYD88
3 lymphoma 32.0 BCL2 BCL6 PAX5
4 lymphoma, hodgkin, classic 30.1 BCL6 PAX5
5 b-cell lymphomas 29.9 BCL2 BCL6 MGMT PAX5
6 intravascular large b-cell lymphoma 29.9 BCL2 BCL6
7 progressive multifocal leukoencephalopathy 29.8 BCL2 IL2
8 lymphocytic leukemia 29.4 BCL2 IL2 MYD88 PAX5
9 lymphoma, non-hodgkin, familial 29.4 BCL2 BCL6 IL2 PAX5
10 leukemia, chronic lymphocytic 29.0 BCL2 BCL6 IGHM IL2 PAX5
11 glioblastoma 10.6
12 acquired immunodeficiency syndrome 10.5
13 glioma 10.4
14 glioblastoma multiforme 10.3
15 multiple sclerosis 10.3
16 hepatitis 10.3
17 meningitis 10.3
18 encephalitis 10.2
19 toxoplasmosis 10.2
20 glioma susceptibility 1 10.2
21 systemic lupus erythematosus 10.2
22 hepatitis b 10.2
23 lupus erythematosus 10.2
24 diabetes insipidus 10.2
25 lymphomatous meningitis 10.2
26 testicular lymphoma 10.2 BCL6 MYD88
27 high grade b-cell lymphoma with myc and/ or bcl2 and/or bcl6 rearrangement 10.2 BCL2 BCL6
28 nodal marginal zone b-cell lymphoma 10.2 BCL2 BCL6
29 follicular lymphoma 1 10.1 BCL2 BCL6
30 central nervous system hematologic cancer 10.1 BCL6 MYD88
31 human herpesvirus 8 10.1 BCL2 BCL6
32 autoimmune lymphoproliferative syndrome, type v 10.1 BCL2 BCL6
33 macroglobulinemia 10.1 MYD88 PAX5
34 gastric lymphoma 10.1 BCL2 BCL6
35 heavy chain disease 10.1 MYD88 PAX5
36 nodular lymphocyte predominant hodgkin lymphoma 10.1 BCL6 PAX5
37 composite lymphoma 10.1 BCL6 PAX5
38 multicentric castleman disease 10.1 BCL6 PAX5
39 rheumatoid arthritis 10.1
40 arthritis 10.1
41 diffuse large b-cell lymphoma 10.1
42 richter's syndrome 10.1
43 hepatitis c 10.1
44 human immunodeficiency virus infectious disease 10.1
45 viral infectious disease 10.1
46 marek disease 10.1
47 encephalopathy 10.1
48 castleman disease 10.1 BCL6 PAX5
49 lung lymphoma 10.1 BCL6 PAX5
50 mediastinal gray zone lymphoma 10.1 CD274 PAX5

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 9.88 BCL2 BCL6 CD274 IL2 MYD88 PAX5
2 hematopoietic system MP:0005397 9.8 BCL2 BCL6 CD274 IL2 MYD88 PAX5
3 endocrine/exocrine gland MP:0005379 9.77 BCL2 BCL6 IL2 MGMT MYD88
4 immune system MP:0005387 9.73 BCL2 BCL6 CD274 IL2 MYD88 PAX5
5 mortality/aging MP:0010768 9.7 BCL2 BCL6 CD274 IL2 MGMT MYD88
6 neoplasm MP:0002006 9.35 BCL2 IL2 MGMT MYD88 PAX5
7 no phenotypic analysis MP:0003012 9.02 BCL6 CD274 IL2 MGMT MYD88

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 250)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
leucovorin Approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 58-05-9 6006 143
2
Methotrexate Approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 59-05-2, 1959-05-2 126941
3
Cytarabine Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 147-94-4 6253
4
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 1177-87-3
5
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 50-02-2 5743
6
Teniposide Approved Phase 4 29767-20-2 34698
7
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 59-30-3 6037
8
Fotemustine Experimental, Investigational Phase 4
9 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
10 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
11 Dermatologic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
12 Vitamin B9 Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
13 Vitamin B Complex Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
14 Folic Acid Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
15 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
16 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
17 Antimetabolites Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
18 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
19 Folate Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
20 Antiviral Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
21 Topoisomerase Inhibitors Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
22 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
23 BB 1101 Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
24
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
25
Levofloxacin Approved, Investigational Phase 3 100986-85-4 149096
26
Ofloxacin Approved Phase 3 82419-36-1 4583
27
Cefepime Approved, Investigational Phase 3 88040-23-7 5479537
28
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1,Not Applicable 22916-47-8 4189
29
rituximab Approved Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable,Early Phase 1 174722-31-7 10201696
30
Temozolomide Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 85622-93-1 5394
31
Carboplatin Approved Phase 3,Phase 1,Phase 2 41575-94-4 10339178 38904 498142
32
Etoposide Approved Phase 3,Phase 1,Phase 2,Not Applicable 33419-42-0 36462
33
Heparin Approved, Investigational Phase 3 9005-49-6 772 46507594
34
Dalteparin Approved Phase 3 9005-49-6
35
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
36
Ondansetron Approved Phase 3,Not Applicable 99614-02-5 4595
37
Caspofungin Approved Phase 3 179463-17-3, 162808-62-0 468682 2826718
38
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
39
Guaifenesin Approved, Investigational, Vet_approved Phase 3,Not Applicable 93-14-1 3516
40
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
41
Morphine Approved, Investigational Phase 3,Not Applicable 57-27-2 5288826
42
Levoleucovorin Approved, Investigational Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable 68538-85-2
43
Idarubicin Approved Phase 2, Phase 3,Phase 3 58957-92-9 42890
44
Mechlorethamine Approved, Investigational Phase 3,Phase 1,Phase 2 51-75-2 4033
45
Thiotepa Approved, Investigational Phase 3,Phase 2 52-24-4 5453
46
Ifosfamide Approved Phase 3,Phase 2,Phase 1 3778-73-2 3690
47
Busulfan Approved, Investigational Phase 3,Phase 2 55-98-1 2478
48
Carmustine Approved, Investigational Phase 3,Phase 2 154-93-8 2578
49
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
50 Pancreatic Polypeptide Investigational Phase 3 59763-91-6

Interventional clinical trials:

(show top 50) (show all 165)
# Name Status NCT ID Phase Drugs
1 Whole Brain Irradiation in Primary Central Nervous System (CNS) Lymphoma (PCNSL) Completed NCT00153530 Phase 4 methotrexate
2 Treatment of Primary CNS Lymphoma Recruiting NCT01960192 Phase 4 HD-MTX-Ara-C regimen;FVD regimen
3 Ketamine Hydrochloride and Best Pain Management in Treating Cancer Patients With Neuropathic Pain Unknown status NCT01316744 Phase 3 ketamine hydrochloride
4 Levofloxacin Compared With Cefepime in Treating Cancer Patients With Fever and Neutropenia Unknown status NCT00020865 Phase 3 cefepime hydrochloride;levofloxacin
5 Dalteparin to Prevent Complications in Cancer Patients Receiving Chemotherapy Through a Catheter Completed NCT00006083 Phase 3 Fragmin
6 Personalized Information or Basic Information in Helping Patients Make Decisions About Participating in a Clinical Trial Completed NCT00750009 Phase 3
7 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
8 St. John's Wort in Relieving Fatigue in Patients Undergoing Chemotherapy or Hormone Therapy for Cancer Completed NCT00005805 Phase 3
9 Ondansetron in Treating Patients With Advanced Cancer and Chronic Nausea and Vomiting Not Caused by Cancer Treatment Completed NCT00006348 Phase 3 ondansetron
10 Caspofungin Acetate Compared With Amphotericin B Liposomal in Treating Patients With Persistent Fever and Neutropenia Following Cancer Treatment Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
11 Treatment for Chronic Pain in Patients With Advanced Cancer Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
12 Therapeutic Effects of R-IDARAM and Intrathecal Immunochemotherapy on Elderly Patients With PCNSL Recruiting NCT02836158 Phase 2, Phase 3 R-IDARAM plus intrathecal chemotherapy
13 BUCYE Conditioning Regimen for PCNSL Undergoing Auto-HSCT Recruiting NCT03733327 Phase 2, Phase 3 Busulfan (BU);Cyclophosphamide (CY);Etoposide (VP-16)
14 Maintenance Treatment Versus Observation in Elderly Patients With PCNS Lymphoma Recruiting NCT02313389 Phase 3 Rituximab, Methotrexate, Temozolomide
15 High-dose Chemotherapy and ASCT or Consolidating Conventional Chemotherapy in Primary CNS Lymphoma Recruiting NCT02531841 Phase 3 Arm A (Fortecortin®-ETOPOPHOS®-IFO-cell®-CARBO-cell®);Arm B (TEPADINA®-CARMUBRIS®-Busilvex®)
16 Treatment of PCNSL With R-IDARAM and Intrathecal Immunochemotherapy Enrolling by invitation NCT02657785 Phase 2, Phase 3 R-IDARAM plus intrathecal chemotherapy
17 a Phase II Study in Primary Central Nervous System Lymphoma Unknown status NCT00455286 Phase 2 Methotrexate;Methylprednisolone;Temozolomide
18 Chemotherapy and Radiation Therapy in Treating Patients With HIV-Related Primary Central Nervous System Lymphoma Unknown status NCT00003261 Phase 2 methotrexate;zidovudine
19 Temsirolimus in Recurrent Primary Central Nervous System (CNS) Lymphoma Unknown status NCT00942747 Phase 2 temsirolimus
20 Pemetrexed and Temozolomide in Treating Patients With Relapsed Primary Central Nervous System Lymphoma (PCNSL) Unknown status NCT01985451 Phase 2 Pemetrexe;Temozolomide
21 Methotrexate and Temozolomide Versus Methotrexate, Procarbazine, Vincristine and Cytarabine Unknown status NCT00503594 Phase 2 Methotrexate and temozolomide;Methotrexate , procarbazine ,vincristine ,cytarabine
22 Temozolomide, Nedaplatin, Vincristine, and Radiotherapy as First-line Treatment in Newly Diagnosed Primary CNS Lymphoma Unknown status NCT01735747 Phase 2 nedaplatin;vincristine;Temozolomide
23 Methotrexate-based Chemo-immunotherapy Followed by Maintenance Therapy for Patients > 65 Years With Central Nervous System (CNS)-Lymphoma Unknown status NCT00989352 Phase 2 Methotrexate;Lomustine;Procarbazine;Rituximab
24 Combination Chemotherapy Plus Filgrastim in Treating Patients With HIV-Related Non-Hodgkin's Lymphoma Unknown status NCT00032149 Phase 1, Phase 2 cyclophosphamide;etoposide;mitoxantrone hydrochloride;prednisolone;vincristine sulfate
25 Phenylbutyrate and Valganciclovir in Treating Patients With Relapsed or Refractory Epstein-Barr Virus-Positive Cancer Unknown status NCT00387530 Phase 2 oral sodium phenylbutyrate;valganciclovir
26 PQR309 in Patients With Relapsed or Refractory Primary Central Nervous System Lymphoma Completed NCT02669511 Phase 2 PQR309
27 Idarubicin Based Combined Modality Therapy in Primary CNS Lymphoma Completed NCT00193973 Phase 2 Idarubicin, Methotrexate, Filgrastim, intrathecal Ara-C
28 Zidovudine Plus Interleukin-2 and Ganciclovir in Treating Patients With AIDS-Related Primary Central Nervous System Lymphoma Completed NCT00006264 Phase 2 ganciclovir;zidovudine
29 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 Trial of Combined Modality Treatment in Primary Central Nervous System Lymphoma Completed NCT00210314 Phase 2 high dose methotrexate;high dose cytarabine
31 Methotrexate, Procarbazine, Lomustine, Dexamethasone, and Cytarabine in Treating Patients With Primary CNS Lymphoma Completed NCT00074191 Phase 2 cytarabine;dexamethasone;lomustine;methotrexate;procarbazine hydrochloride
32 Methotrexate, Cyclophosphamide, and Etoposide Phosphate Given With Osmotic Blood-Brain Barrier Disruption Plus Dexamethasone and Cytarabine in Treating Patients With Primary CNS Lymphoma Completed NCT00074178 Phase 2 cyclophosphamide;cytarabine;dexamethasone;etoposide phosphate;methotrexate
33 Combination Chemotherapy, Monoclonal Antibody, and Radiation Therapy in Treating Patients With 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
34 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
35 Hydroxyurea in Treating Patients With Epstein-Barr Virus-Associated Primary CNS Lymphoma and AIDS Completed NCT00002940 Phase 2 hydroxyurea
36 Combination Chemotherapy Plus Radiation Therapy in Treating Patients With Primary CNS Lymphoma Completed NCT00003061 Phase 2 cytarabine;methotrexate
37 Trial for Patients With Newly Diagnosed Primary Central Nervous System (CNS) Lymphoma Completed NCT01011920 Phase 2 Methotrexate;Ara-C;Rituximab;Thiotepa;BCNU
38 Rituximab and Combination Chemotherapy in Treating Patients With Newly Diagnosed Primary CNS Lymphoma Completed NCT00098774 Phase 2 cytarabine;etoposide;leucovorin calcium;methotrexate;temozolomide
39 Methotrexate and Thiotepa in Treating Patients With Newly Diagnosed Primary CNS Lymphoma Completed NCT00045539 Phase 2 leucovorin calcium;methotrexate;thiotepa
40 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
41 Methotrexate, Glucarpidase, and Leucovorin in Treating Patients With Newly Diagnosed Primary Central Nervous System Lymphoma Completed NCT00727831 Phase 1, Phase 2 glucarpidase;leucovorin calcium;methotrexate
42 High Dose Chemotherapy and Stem Cell Transplant for Non-Hodgkin's Lymphoma or Central Nervous System (CNS) Lymphoma Completed NCT01182415 Phase 2 High-dose chemotherapy
43 Combination Chemotherapy Plus Peripheral Stem Cell Transplantation in Treating Patients With Newly Diagnosed Central Nervous System Lymphoma Completed NCT00003632 Phase 2 carmustine;cytarabine;etoposide;melphalan;methotrexate
44 A Study for Patients With Non-Hodgkin's Lymphomas Completed NCT00542919 Phase 2 enzastaurin
45 Doxorubicin Hydrochloride Liposome and Bortezomib in Treating Patients With Refractory Hematologic Cancer or Malignant Solid Tumor or Metastatic Breast Cancer Completed NCT00237627 Phase 1, Phase 2 PS-341;Doxil;Velcade
46 Peripheral Stem Cell Transplantation in Treating Patients With Breast Cancer or Hematologic Cancer Completed NCT00006225 Phase 1, Phase 2
47 Topotecan Hydrochloride in Treating Children With Meningeal Cancer That Has Not Responded to Previous Treatment Completed NCT00005811 Phase 2 topotecan hydrochloride
48 Combination Chemotherapy in Treating Patients With AIDS-Related Lymphoma 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)
49 Epoetin Alfa or Darbepoetin Alfa in Treating Patients With Anemia Caused by Chemotherapy Completed NCT00416624 Phase 2 darbepoetin alfa;epoetin alfa
50 Epoetin Alfa in Treating Patients With Anemia Who Are Undergoing Chemotherapy for Cancer Completed NCT00255749 Phase 2

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:

41
Brain, B Cells, Eye, Spinal Cord, Liver, T Cells, Bone

Publications for Primary Central Nervous System Lymphoma

Articles related to Primary Central Nervous System Lymphoma:

(show top 50) (show all 914)
# Title Authors Year
1
Intraocular involvement is associated with a high risk of disease relapse in primary central nervous system lymphoma. ( 30320386 )
2019
2
Incidence and survival of primary central nervous system lymphoma (PCNSL): results from the Girona cancer registry (1994-2013). ( 29796994 )
2018
3
Primary central nervous system lymphoma at the cerebellopontine angle mimicking a trigeminal schwannoma: A unique case report and literature review. ( 29656000 )
2018
4
Primary Central Nervous System Lymphoma-PART 1: Epidemiology, Diagnosis, Staging, and Prognosis. ( 29447417 )
2018
5
Significance of treatment response when managing patients with primary central nervous system lymphoma. ( 29966463 )
2018
6
Discrepancy Between Low Levels of mTOR Activity and High Levels of P-S6 in Primary Central Nervous System Lymphoma May Be Explained by PAS Domain-Containing Serine/Threonine-Protein Kinase-Mediated Phosphorylation. ( 29361117 )
2018
7
Primary central nervous system lymphoma in a patient with systemic lupus erythematosus mimicking high-grade glioma: A case report and review of literature. ( 29879076 )
2018
8
Machine Learning-based Texture Analysis of Contrast-enhanced MR Imaging to Differentiate between Glioblastoma and Primary Central Nervous System Lymphoma. ( 29769456 )
2018
9
MyD88 Mutation in Elderly Predicts Poor Prognosis in Primary Central Nervous System Lymphoma: Multi-Institutional Analysis. ( 29258950 )
2018
10
Challenges and prospects in the diagnosis and treatment of primary central nervous system lymphoma. ( 29633883 )
2018
11
Intraoperative Flow Cytometry Enables the Differentiation of Primary Central Nervous System Lymphoma from Glioblastoma. ( 29330078 )
2018
12
Stereotactic brachytherapy with iodine-125 seeds plus temozolomide induce complete and durable remission in a patient with recurrent primary central nervous system lymphoma. ( 29960094 )
2018
13
Population Pharmacokinetics of High-Dose Methotrexate in Patients With Primary Central Nervous System Lymphoma. ( 29331383 )
2018
14
Diffusion radiomics as a diagnostic model for atypical manifestation of primary central nervous system lymphoma: development and multicenter external validation. ( 29438500 )
2018
15
B cell-rich non-neoplastic sentinel lesion preceding primary central nervous system lymphoma. ( 29871654 )
2018
16
Spinal primary central nervous system lymphoma: Case report and literature review. ( 29429785 )
2018
17
Is deep brain involvement in intracranial primary central nervous system lymphoma of importance for penetration of chemotherapeutic agents? ( 29804159 )
2018
18
Primary central nervous system lymphoma presenting as growing intracerebral hemorrhage. ( 29803066 )
2018
19
Isolated acute vestibular syndrome due to presumed primary central nervous system lymphoma involving the dorsal medulla. ( 29936666 )
2018
20
Craniotomy and Survival for Primary Central Nervous System Lymphoma. ( 29660011 )
2018
21
Treatment of newly diagnosed primary central nervous system lymphoma: current and emerging therapies. ( 29741421 )
2018
22
Leakage correction improves prognosis prediction of dynamic susceptibility contrast perfusion MRI in primary central nervous system lymphoma. ( 29323247 )
2018
23
Identification of novel recurrent ETV6-IGH fusions in primary central nervous system lymphoma. ( 29432597 )
2018
24
Phase II study of rituximab given in conjunction with standard chemotherapy in primary central nervous system lymphoma (PCNSL): a trial of the ECOG-ACRIN cancer research group (E1F05). ( 29416652 )
2018
25
Successful rapid drug desensitization to methotrexate in a patient with primary central nervous system lymphoma. ( 29662865 )
2018
26
<sup>18</sup>F-FDG PET/CT in immunocompetent patients with primary central nervous system lymphoma: Differentiation from glioblastoma and correlation with DWI. ( 29857862 )
2018
27
A rare case of paediatric primary central nervous system lymphoma treated with high-dose methotrexate and rituximab-based chemoimmunotherapy and whole brain radiotherapy followed by tumour bed boost with three-dimensional conformal radiation technique. ( 29744624 )
2018
28
A case of primary central nervous system lymphoma arising at the site of remote herpes encephalitis. ( 29382621 )
2018
29
Letter to the Editor Regarding &amp;quot;Natalizumab-Associated Primary Central Nervous System Lymphoma&amp;quot;. ( 29958368 )
2018
30
Molecular analysis in liquid biopsies for diagnostics of primary central nervous system lymphoma: Review of literature and future opportunities. ( 29891112 )
2018
31
Allogeneic stem cell transplantation for relapsed primary central nervous system lymphoma: Is it feasible? ( 29559300 )
2018
32
Novel biomarker, phosphorylated T-LAK cell-originated protein kinase (p-TOPK) can predict outcome in primary central nervous system lymphoma. ( 29575092 )
2018
33
Leukocytoclastic Vasculitis and Desensitization to High-dose Methotrexate in Primary Central Nervous System Lymphoma. ( 29605422 )
2018
34
Primary central nervous system lymphoma and atypical glioblastoma: Differentiation using radiomics approach. ( 29626238 )
2018
35
A prospective phase II trial of response adapted whole brain radiotherapy after high dose methotrexate based chemotherapy in patients with newly diagnosed primary central nervous system lymphoma-analysis of acute toxicity profile and early clinical outcome. ( 29633112 )
2018
36
Prognostic Significance of Interim 11C-Methionine PET/CT in Primary Central Nervous System Lymphoma. ( 29877882 )
2018
37
Target amplicon exome-sequencing identifies promising diagnosis and prognostic markers involved in RTK-RAS and PI3K-AKT signaling as central oncopathways in primary central nervous system lymphoma. ( 29937999 )
2018
38
The diagnostic role and dynamic changes in cerebrospinal fluid neopterin during treatment of patients with primary central nervous system lymphoma. ( 29982995 )
2018
39
Clinical response and pharmacokinetics of bendamustine as a component of salvage R-B(O)AD therapy for the treatment of primary central nervous system lymphoma (PCNSL). ( 29986691 )
2018
40
Novel agents for primary central nervous system lymphoma: evidence and perspectives. ( 29986908 )
2018
41
Impact of histologic subtypes and treatment modality among patients with primary central nervous system lymphoma: a SEER database analysis. ( 29988979 )
2018
42
The expression of PD-1 ligands and IDO1 by macrophage/microglia in primary central nervous system lymphoma. ( 29998979 )
2018
43
The Impact of Early Corticosteroid Pretreatment Before Initiation of Chemotherapy in Patients With Primary Central Nervous System Lymphoma. ( 30016483 )
2018
44
A significant proportion of patients with primary central nervous system lymphoma harbor clonal bone marrow B-cells. ( 30033830 )
2018
45
Prognostic Factors and Survival in Primary Central Nervous System Lymphoma: A Population-Based Study. ( 30050595 )
2018
46
Primary Central Nervous System Lymphoma. ( 30072069 )
2018
47
Pretreatment dynamic contrast-enhanced MRI biomarkers correlate with progression-free survival in primary central nervous system lymphoma. ( 30073640 )
2018
48
Letter: Craniotomy and Survival for Primary Central Nervous System Lymphoma. ( 30085234 )
2018
49
In Reply: Craniotomy and Survival for Primary Central Nervous System Lymphoma. ( 30085238 )
2018
50
Impact of front line relative dose intensity for methotrexate and comorbidities in immunocompetent elderly patients with primary central nervous system lymphoma. ( 30091022 )
2018

Variations for Primary Central Nervous System Lymphoma

Cosmic variations for Primary Central Nervous System Lymphoma:

9 (show all 34)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM10656 TP53 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.742C>T p.R248W 17:7674221-7674221 13
2 COSM43448 TNFAIP3 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.2273C>G p.P758R 6:137881219-137881219 13
3 COSM5878666 TET2 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.4034A>C p.Y1345S 4:105261838-105261838 13
4 COSM4440697 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1255T>A p.S419T 3:177033132-177033132 13
5 COSM4440707 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.596G>A p.S199N 3:177050103-177050103 13
6 COSM4440703 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1376G>A p.S459N 3:177033011-177033011 13
7 COSM4440696 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1256C>T p.S419F 3:177033131-177033131 13
8 COSM4440698 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1335G>T p.V445V 3:177033052-177033052 13
9 COSM4440709 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.148A>T p.I50F 3:177053829-177053829 13
10 COSM4440712 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.230C>T p.P77L 3:177051701-177051701 13
11 COSM4440706 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.637C>A p.H213N 3:177050062-177050062 13
12 COSM4440705 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1274G>T p.R425M 3:177033113-177033113 13
13 COSM4440713 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.229C>A p.P77T 3:177051702-177051702 13
14 COSM4440710 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.70T>C p.S24P 3:177053907-177053907 13
15 COSM4440704 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1336T>G p.Y446D 3:177033051-177033051 13
16 COSM4440708 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.458G>A p.G153E 3:177050580-177050580 13
17 COSM4440695 TBL1XR1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1261G>C p.D421H 3:177033126-177033126 13
18 COSM1716590 STAT5B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1924A>C p.N642H 17:42207711-42207711 13
19 COSM1155745 STAT3 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1981G>C p.D661H 17:42322402-42322402 13
20 COSM97162 PRDM1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.183G>A p.E62fs 6:106088449-106088449 13
21 COSM97163 PRDM1 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.556+1G>C p.? 6:106099553-106099553 13
22 COSM85940 MYD88 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.794T>C p.L265P 3:38141150-38141150 13
23 COSM517 KRAS haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.34G>A p.G12S 12:25245351-25245351 13
24 COSM306058 JAK3 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1533G>T p.M511I 19:17838299-17838299 13
25 COSM5878665 DNMT3A haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.2207G>T p.R736L 2:25240417-25240417 13
26 COSM144393 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.586T>A p.Y196N 17:63929439-63929439 13
27 COSM220733 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.587A>C p.Y196S 17:63929438-63929438 13
28 COSM1737939 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.586T>G p.Y196D 17:63929439-63929439 13
29 COSM220734 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.586T>C p.Y196H 17:63929439-63929439 13
30 COSM220736 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.587A>G p.Y196C 17:63929438-63929438 13
31 COSM5045028 CD79B haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.591G>C p.E197D 17:63929434-63929434 13
32 COSM43455 CARD11 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.343T>C p.F115L 7:2945834-2945834 13
33 COSM43452 CARD11 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.343T>A p.F115I 7:2945834-2945834 13
34 COSM43451 CARD11 haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,primary central nervous system lymphoma c.1010G>A p.R337Q 7:2938686-2938686 13

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

7 (show top 50) (show all 54)
# CNVD ID Chromosom 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 GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.54 BCL6 CD274 IL2 MYD88 PAX5
2
Show member pathways
11.19 BCL2 IL2
3 11.05 BCL6 PAX5
4 10.96 BCL2 IL2
5
Show member pathways
10.8 BCL2 MGMT
6 10.37 BCL2 IL2

GO Terms for Primary Central Nervous System Lymphoma

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.71 BCL6 IGHM IL2 MYD88
2 B cell differentiation GO:0030183 9.55 BCL2 BCL6
3 cell morphogenesis GO:0000902 9.54 BCL2 BCL6
4 regulation of inflammatory response GO:0050727 9.52 BCL6 MYD88
5 positive regulation of T cell proliferation GO:0042102 9.51 CD274 IL2
6 response to cytokine GO:0034097 9.49 BCL2 CD274
7 humoral immune response GO:0006959 9.48 BCL2 PAX5
8 T cell differentiation GO:0030217 9.46 BCL2 IL2
9 cytokine-mediated signaling pathway GO:0019221 9.46 BCL2 BCL6 IL2 MYD88
10 extrinsic apoptotic signaling pathway in absence of ligand GO:0097192 9.43 BCL2 IL2
11 positive regulation of interleukin-17 production GO:0032740 9.37 IL2 MYD88
12 negative regulation of apoptotic process GO:0043066 9.35 BCL2 BCL6 IL2 MGMT MYD88
13 positive regulation of regulatory T cell differentiation GO:0045591 9.32 BCL6 IL2
14 negative regulation of B cell apoptotic process GO:0002903 9.26 BCL6 IL2
15 positive regulation of B cell proliferation GO:0030890 8.8 BCL2 BCL6 IL2

Sources for Primary Central Nervous System Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
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49 NCI
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51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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