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Diffuse large B-cell lymphoma of the central nervous system

MedGen UID:
378339
•Concept ID:
C2026186
•
Neoplastic Process
Synonyms: Central Nervous System Diffuse Large B-Cell Lymphoma; central nervous system diffuse large B-cell lymphoma; CNS DLBCL; Diffuse large B-cell lymphoma of central nervous system; diffuse large B-cell lymphoma of central nervous system; diffuse large B-cell lymphoma of the central nervous system; DLBCL of the CNS; Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System; primary diffuse large B-cell lymphoma of the central nervous system; Primary DLBCL of the CNS; primary DLBCL of the CNS
SNOMED CT: Diffuse large B-cell lymphoma of central nervous system (734066005)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017596
Orphanet: ORPHA300849

Definition

A diffuse large B-cell lymphoma arising from the central nervous system. [from NCI]

Professional guidelines

PubMed

Patient-derived xenograft mouse models to investigate tropism to the central nervous system and retina of primary and secondary central nervous system lymphoma.
Isbell LK, Tschuch C, Doostkam S, Waldeck S, Andrieux G, Shoumariyeh K, Lenhard D, Schaefer HE, Reinacher PC, Bartsch I, Pantic M, Vinnakota JM, Kakkassery V, Schorb E, Scherer F, Frey AV, Boerries M, Illerhaus G, Duyster J, Schueler J, von Bubnoff N
Neuropathol Appl Neurobiol 2023 Apr;49(2):e12899. doi: 10.1111/nan.12899. PMID: 36879456
Correlation study of PD-L1, CD4, CD8, and PD-1 in primary diffuse large B-cell lymphoma of the central nervous system.
Liu B, Yan S, Li S, Zhang Q, Yang M, Yang L, Ma J, Li X
Pathol Res Pract 2022 Nov;239:154008. Epub 2022 Aug 23 doi: 10.1016/j.prp.2022.154008. PMID: 36183436
Induction therapy with the MATRix regimen in patients with newly diagnosed primary diffuse large B-cell lymphoma of the central nervous system - an international study of feasibility and efficacy in routine clinical practice.
Schorb E, Fox CP, Kasenda B, Linton K, Martinez-Calle N, Calimeri T, Ninkovic S, Eyre TA, Cummin T, Smith J, Yallop D, De Marco B, Krampera M, Trefz S, Orsucci L, Fabbri A, Illerhaus G, Cwynarski K, Ferreri AJM
Br J Haematol 2020 Jun;189(5):879-887. Epub 2020 Jan 29 doi: 10.1111/bjh.16451. PMID: 31997308

Recent clinical studies

Etiology

Clinicopathological features of primary diffuse large B-cell lymphoma of the central nervous system - strong EZH2 expression implying diagnostic and therapeutic implication.
Guo S, Bai Q, Rohr J, Wang Y, Liu Y, Zeng K, Yu K, Zhang X, Wang Z
APMIS 2016 Dec;124(12):1054-1062. Epub 2016 Nov 2 doi: 10.1111/apm.12623. PMID: 27807891

Diagnosis

Induction therapy with the MATRix regimen in patients with newly diagnosed primary diffuse large B-cell lymphoma of the central nervous system - an international study of feasibility and efficacy in routine clinical practice.
Schorb E, Fox CP, Kasenda B, Linton K, Martinez-Calle N, Calimeri T, Ninkovic S, Eyre TA, Cummin T, Smith J, Yallop D, De Marco B, Krampera M, Trefz S, Orsucci L, Fabbri A, Illerhaus G, Cwynarski K, Ferreri AJM
Br J Haematol 2020 Jun;189(5):879-887. Epub 2020 Jan 29 doi: 10.1111/bjh.16451. PMID: 31997308
Clinicopathological features of primary diffuse large B-cell lymphoma of the central nervous system - strong EZH2 expression implying diagnostic and therapeutic implication.
Guo S, Bai Q, Rohr J, Wang Y, Liu Y, Zeng K, Yu K, Zhang X, Wang Z
APMIS 2016 Dec;124(12):1054-1062. Epub 2016 Nov 2 doi: 10.1111/apm.12623. PMID: 27807891

Therapy

Induction therapy with the MATRix regimen in patients with newly diagnosed primary diffuse large B-cell lymphoma of the central nervous system - an international study of feasibility and efficacy in routine clinical practice.
Schorb E, Fox CP, Kasenda B, Linton K, Martinez-Calle N, Calimeri T, Ninkovic S, Eyre TA, Cummin T, Smith J, Yallop D, De Marco B, Krampera M, Trefz S, Orsucci L, Fabbri A, Illerhaus G, Cwynarski K, Ferreri AJM
Br J Haematol 2020 Jun;189(5):879-887. Epub 2020 Jan 29 doi: 10.1111/bjh.16451. PMID: 31997308

Prognosis

Clinicopathological features of primary diffuse large B-cell lymphoma of the central nervous system - strong EZH2 expression implying diagnostic and therapeutic implication.
Guo S, Bai Q, Rohr J, Wang Y, Liu Y, Zeng K, Yu K, Zhang X, Wang Z
APMIS 2016 Dec;124(12):1054-1062. Epub 2016 Nov 2 doi: 10.1111/apm.12623. PMID: 27807891

Clinical prediction guides

Clinicopathological features of primary diffuse large B-cell lymphoma of the central nervous system - strong EZH2 expression implying diagnostic and therapeutic implication.
Guo S, Bai Q, Rohr J, Wang Y, Liu Y, Zeng K, Yu K, Zhang X, Wang Z
APMIS 2016 Dec;124(12):1054-1062. Epub 2016 Nov 2 doi: 10.1111/apm.12623. PMID: 27807891

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