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Primary central nervous system lymphoma

MedGen UID:
79187
•Concept ID:
C0280803
•
Neoplastic Process
Synonyms: Central Nervous System Lymphoma; central nervous system lymphoma; CNS Lymphoma; CNS lymphoma; lymphoma of central nervous system; Malignant Lymphomas of CNS; malignant lymphomas of CNS; Malignant Lymphomas of the CNS; malignant lymphomas of the CNS; Microglioma; microglioma; PCNSL; PCNSL - Primary CNS lymphoma; Primary brain lymphoma; primary brain lymphoma; Primary Central Nervous System Lymphoma; primary central nervous system lymphoma; Primary CNS Lymphoma; Primary CNS lymphoma; primary CNS lymphoma; primary lymphoma, CNS
SNOMED CT: Primary central nervous system lymphoma (307649006); Microglioma (307649006); Primary CNS lymphoma (307649006); PCNSL - Primary CNS lymphoma (307649006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0030069
Monarch Initiative: MONDO:0002571
Orphanet: ORPHA46135

Definition

A form of extranodal, high-grade non-Hodgkin B-cell neoplasm, usually large cell or immunoblastic type that originates in the brain, leptomeninges, spinal cord, or eyes and typically remains confined to the CNS. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary central nervous system lymphoma
Follow this link to review classifications for Primary central nervous system lymphoma in Orphanet.

Professional guidelines

PubMed

Primary central nervous system lymphoma.
Ferreri AJM, Calimeri T, Cwynarski K, Dietrich J, Grommes C, Hoang-Xuan K, Hu LS, Illerhaus G, Nayak L, Ponzoni M, Batchelor TT
Nat Rev Dis Primers 2023 Jun 15;9(1):29. doi: 10.1038/s41572-023-00439-0. PMID: 37322012Free PMC Article
Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
Comprehensive approach to diagnosis and treatment of newly diagnosed primary CNS lymphoma.
Grommes C, Rubenstein JL, DeAngelis LM, Ferreri AJM, Batchelor TT
Neuro Oncol 2019 Feb 19;21(3):296-305. doi: 10.1093/neuonc/noy192. PMID: 30418592Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Central Nervous System Cancers, 2023

Recent clinical studies

Etiology

Primary Central Nervous System Lymphomas.
Chukwueke U, Grommes C, Nayak L
Hematol Oncol Clin North Am 2022 Feb;36(1):147-159. doi: 10.1016/j.hoc.2021.09.004. PMID: 34801161
STAT6: A review of a signaling pathway implicated in various diseases with a special emphasis in its usefulness in pathology.
Karpathiou G, Papoudou-Bai A, Ferrand E, Dumollard JM, Peoc'h M
Pathol Res Pract 2021 Jul;223:153477. Epub 2021 May 11 doi: 10.1016/j.prp.2021.153477. PMID: 33991851
Consensus recommendations for MRI and PET imaging of primary central nervous system lymphoma: guideline statement from the International Primary CNS Lymphoma Collaborative Group (IPCG).
Barajas RF, Politi LS, Anzalone N, Schöder H, Fox CP, Boxerman JL, Kaufmann TJ, Quarles CC, Ellingson BM, Auer D, Andronesi OC, Ferreri AJM, Mrugala MM, Grommes C, Neuwelt EA, Ambady P, Rubenstein JL, Illerhaus G, Nagane M, Batchelor TT, Hu LS
Neuro Oncol 2021 Jul 1;23(7):1056-1071. doi: 10.1093/neuonc/noab020. PMID: 33560416Free PMC Article
Updates on Primary Central Nervous System Lymphoma.
Schaff LR, Grommes C
Curr Oncol Rep 2018 Feb 28;20(2):11. doi: 10.1007/s11912-018-0666-1. PMID: 29492682Free PMC Article
Natalizumab-Associated Primary Central Nervous System Lymphoma.
Nixon M, Menger RP, Kalakoti P, Thakur JD, Dossani RH, Sharma K, Nanda A, Guthikonda B
World Neurosurg 2018 Jan;109:152-159. Epub 2017 Sep 28 doi: 10.1016/j.wneu.2017.09.131. PMID: 28962961

Diagnosis

Primary central nervous system lymphoma.
Ferreri AJM, Calimeri T, Cwynarski K, Dietrich J, Grommes C, Hoang-Xuan K, Hu LS, Illerhaus G, Nayak L, Ponzoni M, Batchelor TT
Nat Rev Dis Primers 2023 Jun 15;9(1):29. doi: 10.1038/s41572-023-00439-0. PMID: 37322012Free PMC Article
Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
Intratumor heterogeneity and T cell exhaustion in primary CNS lymphoma.
Heming M, Haessner S, Wolbert J, Lu IN, Li X, Brokinkel B, Müther M, Holling M, Stummer W, Thomas C, Schulte-Mecklenbeck A, de Faria F, Stoeckius M, Hailfinger S, Lenz G, Kerl K, Wiendl H, Meyer Zu Hörste G, Grauer OM
Genome Med 2022 Sep 24;14(1):109. doi: 10.1186/s13073-022-01110-1. PMID: 36153593Free PMC Article
Primary central nervous system lymphoma.
Schaff LR, Grommes C
Blood 2022 Sep 1;140(9):971-979. doi: 10.1182/blood.2020008377. PMID: 34699590Free PMC Article
Diagnosis and management of primary central nervous system lymphoma.
Han CH, Batchelor TT
Cancer 2017 Nov 15;123(22):4314-4324. Epub 2017 Sep 26 doi: 10.1002/cncr.30965. PMID: 28950405

Therapy

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
Primary Central Nervous System Lymphomas.
Chukwueke U, Grommes C, Nayak L
Hematol Oncol Clin North Am 2022 Feb;36(1):147-159. doi: 10.1016/j.hoc.2021.09.004. PMID: 34801161
Primary central nervous system lymphoma.
Schaff LR, Grommes C
Blood 2022 Sep 1;140(9):971-979. doi: 10.1182/blood.2020008377. PMID: 34699590Free PMC Article
Consensus recommendations for MRI and PET imaging of primary central nervous system lymphoma: guideline statement from the International Primary CNS Lymphoma Collaborative Group (IPCG).
Barajas RF, Politi LS, Anzalone N, Schöder H, Fox CP, Boxerman JL, Kaufmann TJ, Quarles CC, Ellingson BM, Auer D, Andronesi OC, Ferreri AJM, Mrugala MM, Grommes C, Neuwelt EA, Ambady P, Rubenstein JL, Illerhaus G, Nagane M, Batchelor TT, Hu LS
Neuro Oncol 2021 Jul 1;23(7):1056-1071. doi: 10.1093/neuonc/noab020. PMID: 33560416Free PMC Article
Phase I/II study of tirabrutinib, a second-generation Bruton's tyrosine kinase inhibitor, in relapsed/refractory primary central nervous system lymphoma.
Narita Y, Nagane M, Mishima K, Terui Y, Arakawa Y, Yonezawa H, Asai K, Fukuhara N, Sugiyama K, Shinojima N, Kitagawa J, Aoi A, Nishikawa R
Neuro Oncol 2021 Jan 30;23(1):122-133. doi: 10.1093/neuonc/noaa145. PMID: 32583848Free PMC Article

Prognosis

STAT6: A review of a signaling pathway implicated in various diseases with a special emphasis in its usefulness in pathology.
Karpathiou G, Papoudou-Bai A, Ferrand E, Dumollard JM, Peoc'h M
Pathol Res Pract 2021 Jul;223:153477. Epub 2021 May 11 doi: 10.1016/j.prp.2021.153477. PMID: 33991851
Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (Review).
Liu Y, Yao Q, Zhang F
Int J Oncol 2021 Mar;58(3):371-387. Epub 2021 Feb 1 doi: 10.3892/ijo.2021.5180. PMID: 33650642Free PMC Article
Central Nervous System Lymphoma.
Chukwueke UN, Nayak L
Hematol Oncol Clin North Am 2019 Aug;33(4):597-611. Epub 2019 May 10 doi: 10.1016/j.hoc.2019.03.008. PMID: 31229157
Comprehensive approach to diagnosis and treatment of newly diagnosed primary CNS lymphoma.
Grommes C, Rubenstein JL, DeAngelis LM, Ferreri AJM, Batchelor TT
Neuro Oncol 2019 Feb 19;21(3):296-305. doi: 10.1093/neuonc/noy192. PMID: 30418592Free PMC Article
R-MPV followed by high-dose chemotherapy with TBC and autologous stem-cell transplant for newly diagnosed primary CNS lymphoma.
Omuro A, Correa DD, DeAngelis LM, Moskowitz CH, Matasar MJ, Kaley TJ, Gavrilovic IT, Nolan C, Pentsova E, Grommes CC, Panageas KS, Baser RE, Faivre G, Abrey LE, Sauter CS
Blood 2015 Feb 26;125(9):1403-10. Epub 2015 Jan 7 doi: 10.1182/blood-2014-10-604561. PMID: 25568347Free PMC Article

Clinical prediction guides

Toxicity and efficacy of CAR T-cell therapy in primary and secondary CNS lymphoma: a meta-analysis of 128 patients.
Cook MR, Dorris CS, Makambi KH, Luo Y, Munshi PN, Donato M, Rowley S, Saad A, Goy A, Dunleavy K, Ali A
Blood Adv 2023 Jan 10;7(1):32-39. doi: 10.1182/bloodadvances.2022008525. PMID: 36260735Free PMC Article
STAT6: A review of a signaling pathway implicated in various diseases with a special emphasis in its usefulness in pathology.
Karpathiou G, Papoudou-Bai A, Ferrand E, Dumollard JM, Peoc'h M
Pathol Res Pract 2021 Jul;223:153477. Epub 2021 May 11 doi: 10.1016/j.prp.2021.153477. PMID: 33991851
Outcomes Associated With Thiotepa-Based Conditioning in Patients With Primary Central Nervous System Lymphoma After Autologous Hematopoietic Cell Transplant.
Scordo M, Wang TP, Ahn KW, Chen Y, Ahmed S, Awan FT, Beitinjaneh A, Chen A, Chow VA, Dholaria B, Epperla N, Farooq U, Ghosh N, Grover N, Hamad N, Hildebrandt GC, Holmberg L, Hong S, Inwards DJ, Jimenez-Jimenez A, Karmali R, Kenkre VP, Khimani F, Klyuchnikov E, Krem MM, Munshi PN, Nieto Y, Prestidge T, Ramakrishnan Geethakumari P, Rezvani AR, Riedell PA, Seo S, Shah NN, Solh M, Yared JA, Kharfan-Dabaja MA, Herrera A, Hamadani M, Sauter CS
JAMA Oncol 2021 Jul 1;7(7):993-1003. doi: 10.1001/jamaoncol.2021.1074. PMID: 33956047Free PMC Article
Phase I/II study of tirabrutinib, a second-generation Bruton's tyrosine kinase inhibitor, in relapsed/refractory primary central nervous system lymphoma.
Narita Y, Nagane M, Mishima K, Terui Y, Arakawa Y, Yonezawa H, Asai K, Fukuhara N, Sugiyama K, Shinojima N, Kitagawa J, Aoi A, Nishikawa R
Neuro Oncol 2021 Jan 30;23(1):122-133. doi: 10.1093/neuonc/noaa145. PMID: 32583848Free PMC Article
R-MPV followed by high-dose chemotherapy with TBC and autologous stem-cell transplant for newly diagnosed primary CNS lymphoma.
Omuro A, Correa DD, DeAngelis LM, Moskowitz CH, Matasar MJ, Kaley TJ, Gavrilovic IT, Nolan C, Pentsova E, Grommes CC, Panageas KS, Baser RE, Faivre G, Abrey LE, Sauter CS
Blood 2015 Feb 26;125(9):1403-10. Epub 2015 Jan 7 doi: 10.1182/blood-2014-10-604561. PMID: 25568347Free PMC Article

Recent systematic reviews

Role of Rituximab in Treatment of Patients With Primary Central Nervous System Lymphoma (PCNSL): A Systematic Review and Meta-Analysis.
Zhang Y, Liu Z, Gao C, Bian H, Ma Y, Jing F, Zhao X
Clin Lymphoma Myeloma Leuk 2023 Oct;23(10):733-741. Epub 2023 Jun 26 doi: 10.1016/j.clml.2023.06.008. PMID: 37453867
Toxicity and efficacy of CAR T-cell therapy in primary and secondary CNS lymphoma: a meta-analysis of 128 patients.
Cook MR, Dorris CS, Makambi KH, Luo Y, Munshi PN, Donato M, Rowley S, Saad A, Goy A, Dunleavy K, Ali A
Blood Adv 2023 Jan 10;7(1):32-39. doi: 10.1182/bloodadvances.2022008525. PMID: 36260735Free PMC Article
HIV prevalence in primary central nervous system lymphoma: A systematic review and meta-analysis.
Franca RA, Travaglino A, Varricchio S, Russo D, Picardi M, Pane F, Pace M, Del Basso De Caro M, Mascolo M
Pathol Res Pract 2020 Nov;216(11):153192. Epub 2020 Sep 6 doi: 10.1016/j.prp.2020.153192. PMID: 32956921
Rituximab in primary central nervous system lymphoma-A systematic review and meta-analysis.
Schmitt AM, Herbrand AK, Fox CP, Bakunina K, Bromberg JEC, Cwynarski K, Doorduijn JK, Ferreri AJM, Illerhaus G, Issa S, Schorb E, Zucca E, Hemkens LG, Schandelmaier S, Kasenda B
Hematol Oncol 2019 Dec;37(5):548-557. Epub 2019 Oct 9 doi: 10.1002/hon.2666. PMID: 31418878
Natalizumab-Associated Primary Central Nervous System Lymphoma.
Nixon M, Menger RP, Kalakoti P, Thakur JD, Dossani RH, Sharma K, Nanda A, Guthikonda B
World Neurosurg 2018 Jan;109:152-159. Epub 2017 Sep 28 doi: 10.1016/j.wneu.2017.09.131. PMID: 28962961

Supplemental Content

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    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Central Nervous System Cancers, 2023

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
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      Related literature resources in PubMed

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