PMID- 35406516
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220415
IS  - 2072-6694 (Print)
IS  - 2072-6694 (Electronic)
IS  - 2072-6694 (Linking)
VI  - 14
IP  - 7
DP  - 2022 Mar 29
TI  - Extranodal Marginal Zone Lymphoma: Pathogenesis, Diagnosis and Treatment.
LID - 10.3390/cancers14071742 [doi]
LID - 1742
AB  - Extranodal Marginal Zone Lymphoma (EMZL lymphoma) is an indolent B-cell lymphoma 
      with a median age at diagnosis of about 60 years. It accounts for 7-8% of all 
      B-cell lymphomas. It can occur in various extranodal sites, including stomach, 
      lung, ocular adnexa, and skin; furthermore, the disseminated disease can be found 
      in 25-50% of cases. Several infectious agents, such as Helicobacter pylori (H. 
      Pylori) in the case of gastric Mucosa Associated Lymphoid Tissue (MALT) Lymphoma, 
      can drive the pathogenesis of this cancer, through the autoantigenic stimulation 
      of T cells, but there may also be other factors participating such autoimmune 
      diseases. Initial staging should include total body computed tomography, bone 
      marrow aspirate, and endoscopic investigation if indicated. Fluorescence in situ 
      hybridization (FISH), should be performed to detect the presence of specific 
      chromosomal translocations involving the MALT1 and BCL10 genes, which leads to 
      the activation of the NF-kappaB signaling pathway. Depending on the location and 
      dissemination of the disease, different therapeutic choices may include targeted 
      therapy against the etiopathogenetic agent, radiotherapy, immunochemotherapy, and 
      biological drugs. The purpose of this review is to illustrate the complex biology 
      and the diagnosis of this disease and to better define new treatment strategies.
FAU - Di Rocco, Alice
AU  - Di Rocco A
AD  - Hematology, Departement of Traslational and Precision Medicine, Sapienza 
      University of Rome, 00161 Rome, Italy.
FAU - Petrucci, Luigi
AU  - Petrucci L
AD  - Hematology, Departement of Traslational and Precision Medicine, Sapienza 
      University of Rome, 00161 Rome, Italy.
FAU - Assanto, Giovanni Manfredi
AU  - Assanto GM
AUID- ORCID: 0000-0002-6190-9635
AD  - Hematology, Departement of Traslational and Precision Medicine, Sapienza 
      University of Rome, 00161 Rome, Italy.
FAU - Martelli, Maurizio
AU  - Martelli M
AD  - Hematology, Departement of Traslational and Precision Medicine, Sapienza 
      University of Rome, 00161 Rome, Italy.
FAU - Pulsoni, Alessandro
AU  - Pulsoni A
AUID- ORCID: 0000-0001-7429-8004
AD  - Hematology, Departement of Traslational and Precision Medicine, Sapienza 
      University of Rome, 00161 Rome, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20220329
PL  - Switzerland
TA  - Cancers (Basel)
JT  - Cancers
JID - 101526829
PMC - PMC8997163
OTO - NOTNLM
OT  - BALT
OT  - MALT
OT  - OAL
OT  - diagnosis
OT  - immunotherapy
OT  - marginal zone lymphoma
OT  - non-Hodgkin lymphoma
OT  - prognosis
OT  - targeted-therapy
OT  - treatment
COIS- The authors declare no conflict of interest.
EDAT- 2022/04/13 06:00
MHDA- 2022/04/13 06:01
PMCR- 2022/03/29
CRDT- 2022/04/12 01:04
PHST- 2022/02/02 00:00 [received]
PHST- 2022/03/25 00:00 [revised]
PHST- 2022/03/25 00:00 [accepted]
PHST- 2022/04/12 01:04 [entrez]
PHST- 2022/04/13 06:00 [pubmed]
PHST- 2022/04/13 06:01 [medline]
PHST- 2022/03/29 00:00 [pmc-release]
AID - cancers14071742 [pii]
AID - cancers-14-01742 [pii]
AID - 10.3390/cancers14071742 [doi]
PST - epublish
SO  - Cancers (Basel). 2022 Mar 29;14(7):1742. doi: 10.3390/cancers14071742.