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Titel: Management of Extranodal Marginal Zone Lymphoma: Present and Upcoming Perspectives
VerfasserIn: Kaddu-Mulindwa, Dominic
Thurner, Lorenz
Christofyllakis, Konstantinos
Bewarder, Moritz
Kos, Igor Age
Sprache: Englisch
Titel: Cancers
Bandnummer: 14
Heft: 12
Verlag/Plattform: MDPI
Erscheinungsjahr: 2022
Freie Schlagwörter: marginal zone cell lymphoma
MALT
management
indolent lymphoma
DDC-Sachgruppe: 610 Medizin, Gesundheit
Dokumenttyp: Journalartikel / Zeitschriftenartikel
Abstract: Extranodal marginal zone lymphoma (EMZL) encompasses a subgroup of non-Hodgkin lymphomas that often present with localized involvement and may manifest in a diversity of organs and tissues. EMZL pathogenesis is in some cases linked to chronic inflammation/infection, which may impose additional diagnostic and clinical challenges. The most studied and established connection is the presence of Helicobacter pylori in gastric EMZL. Due to its heterogeneity of presentation and intricate pathological features, treatment can be complex, and staging systems are decisive for the choice of therapy. Nevertheless, there is no consensus regarding the most suitable staging system, and recommendations vary among different countries. As a rule of thumb, in limited stages, a local therapy with surgery or radiation is the preferred option, and it is potentially curative. Of note, eradicating the causal agent may be an important step of treatment, especially in gastric EMZL, in which Helicobacter pylori eradication remains the first-line therapy for the majority of patients. In patients with more advanced stages, watch-and-wait is a valuable option, especially amongst those without clear indications for systemic therapy, and it may be carried on for several years. If watch-and-wait is not an option, systemic therapy may be needed. Even though several agents have been tested as monotherapy or in combination in recent years, there is no consensus regarding the first-line therapy, and decisions can vary depending on individual factors, such as age, clinical performance and stage. This review aims to discuss the several aspects of EMZL, including genetic milieu, pathogenesis and staging systems, that may influence the choice of therapy. In addition, we present a summary of evidence of several systemic therapies, compare different recommendations worldwide and discuss future perspectives and novelties in its therapy.
DOI der Erstveröffentlichung: 10.3390/cancers14123019
Link zu diesem Datensatz: urn:nbn:de:bsz:291--ds-365745
hdl:20.500.11880/33226
http://dx.doi.org/10.22028/D291-36574
ISSN: 2072-6694
Datum des Eintrags: 24-Jun-2022
Fakultät: M - Medizinische Fakultät
Fachrichtung: M - Innere Medizin
Professur: M - Prof. Dr. Stephan Stilgenbauer
M - Dr. med. Lorenz Thurner
Sammlung:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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