title: Update on the molecular pathogenesis and targeted approaches of mantle cell lymphoma: summary of the 12th annual conference of the European Mantle Cell Lymphoma Network.
abstract: Mantle cell lymphoma (MCL) is a distinct subtype of malignant lymphoma which is characterized by the chromosomal translocation t(11;14)(q13;q32) resulting in constitutional overexpression of cyclin D1 and cell cycle dysregulation in almost all cases. Clinically, MCL shows an aggressive clinical course with a continuous relapse pattern and a median survival of only 3-5 years. However, recently a subset of 15% long-term survivors has been identified with a rather indolent clinical course. Targeted strategies include the proteasome inhibitors, immune modulatory drugs (IMiDs), mammalian target of rapamycin (mTOR) inhibitors and especially inhibitors of the B-cell receptor pathway. Our recent annual conference focused on the molecular pathogenesis of the disease and how these underlying molecular alterations may guide the selection and integration of innovative approaches for therapy. This review of the meeting covers in particular the identification of indolent cases, and deals with the role of the B-cell receptor pathway in MCL, as well as the detection of minimal residual disease and implementation of molecular approaches in current clinical trials.
authors.affiliation: Department of Medicine III, University Hospital/LMU Munich , Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Dreyling
authors.forename: Virginia
authors.initials: V
authors.lastname: Amador
authors.forename: Mary
authors.initials: M
authors.lastname: Callanan
authors.forename: Mats
authors.initials: M
authors.lastname: Jerkeman
authors.forename: Steven
authors.initials: S
authors.lastname: Le Gouill
authors.forename: Christiane
authors.initials: C
authors.lastname: Pott
authors.forename: Simon
authors.initials: S
authors.lastname: Rule
authors.forename: Francesco
authors.initials: F
authors.lastname: Zaja
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000235:genetics* / Q000628:therapy*; D058990:Molecular Targeted Therapy / Q000379:methods* / Q000639:trends; D018365:Neoplasm, Residual / Q000175:diagnosis / Q000235:genetics; D033581:Stem Cell Transplantation / Q000379:methods*; D016019:Survival Analysis; D014178:Translocation, Genetic; D016896:Treatment Outcome
keywords: Mantle cell lymphoma; chemotherapy; minimal residual disease; molecular pathogenesis; targeted therapy