title: Dual ALK and MYC rearrangements leading to an aggressive variant of anaplastic large cell lymphoma.
abstract: Anaplastic lymphoma kinase (ALK) and MYC are oncogenes often dysregulated in pediatric lymphomas. NPM-ALK/t(2;5)(p23;q35) is a genetic hallmark of ALK anaplastic large cell lymphoma (ALCL). MYC gene translocations are frequently detected in high-grade B-cell lymphomas. ALKALCL cases with concurrent MYC translocation are exceedingly rare and are more aggressive and chemoresistent compared with other ALKALCL. We report a patient who presented with ALKALCL possessing coexistent MYC rearrangement, massive tumor dissemination, and early widespread relapse. This case underscores the importance of recognition of close correlation between dual ALK and MYC rearrangements and the characteristic clinical features in this unusual ALCL variant.
authors.affiliation: Department of Pathology, Children's Hospital Colorado, Aurora, CO 80045, USA. liang.xiayuan@tchden.org
authors.forename: Xiayuan
authors.initials: X
authors.lastname: Liang
authors.forename: Brian
authors.initials: B
authors.lastname: Branchford
authors.forename: Brian
authors.initials: B
authors.lastname: Greffe
authors.forename: Loris
authors.initials: L
authors.lastname: McGavran
authors.forename: Billie
authors.initials: B
authors.lastname: Carstens
authors.forename: Lynne
authors.initials: L
authors.lastname: Meltesen
authors.forename: Edith A
authors.initials: EA
authors.lastname: Albano
authors.forename: Ralph
authors.initials: R
authors.lastname: Quinones
authors.forename: Bruce
authors.initials: B
authors.lastname: Cook
authors.forename: Douglas K
authors.initials: DK
authors.lastname: Graham
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D015321:Gene Rearrangement; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics*; D014178:Translocation, Genetic
chemical_list: D015514:Oncogene Proteins, Fusion; D016271:Proto-Oncogene Proteins c-myc; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases