Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12857/115795
Title: Anaplastic lymphoma kinase aberrations correlate with metastatic features in pediatric rhabdomyosarcoma
Authors: Gasparini, Patrizia
Casanova, Michela
Villa, Raffaella
Collini, Paola
Alaggio, Rita 
Zin, Angelica 
Bonvini, Paolo
Antonescu, Cristina R
Boldrini, Renata
Caserini, Roberto
Moro, Massimo
Centonze, Giovanni
Meazza, Cristina
Massimino, Maura
Bergamaschi, Luca
Luksch, Roberto
Chiaravalli, Stefano
Bisogno, Gianni 
Zaffaroni, Nadia
Daidone, Maria Grazia
Sozzi, Gabriella
Ferrari, Andrea
Keywords: EML4-ALK;anaplastic lymphoma kinase;chromosomal rearrangement;metastasis;rhabdomyosarcoma
Keywords Plus: SOFT-TISSUE SARCOMA;FUSION GENE;ALK;EXPRESSION;IDENTIFICATION;TRANSLOCATION;PAX3
Mesh headings: Mesenchymal Stem Cells;Muscle, Skeletal;Receptor Protein-Tyrosine Kinases;Rhabdomyosarcoma
Secondary Mesh headings: Adolescent;Adult;Anaplastic Lymphoma Kinase;Cell Differentiation;Cell Line, Tumor;Child;Child, Preschool;Cohort Studies;Female;Gene Rearrangement;Humans;Infant;Infant, Newborn;Male;Neoplasm Metastasis;RNA, Small Interfering;Treatment Outcome;Young Adult
Issue Date: 2016
Publisher: IMPACT JOURNALS LLC
Journal: Oncotarget 
Abstract: 
Rhabdomyosarcoma (RMS) is the most frequent soft tissue tumor in childhood and arises from immature mesenchymal cells committed to skeletal muscle differentiation. Anaplastic Lymphoma Kinase (ALK) is a receptor tyrosine kinase aberrantly expressed in several cancers. Moreover, ALK full-length receptor protein has been observed in RMS, although its clinical and functional significance is yet controversial. The role of ALK and its clinical relevance were investigated in a selected cohort of 74 FFPE pediatric RMS and a panel of RMS cell lines, evaluating its gene and protein status, utilizing Fluorescent In Situ Hybridization (FISH), immunohistochemistry (IHC) and Western blot approaches. Moreover, to get insight into its possible therapeutic relevance, effects of ALK silencing on cell proliferation, invasion and apoptosis were studied in RMS cells. ALK IHC positivity was significantly correlated with gene copy number gain, the alveolar subtype, PAX3/7-FOXO1 rearrangements, the presence of metastasis at diagnosis and a worse overall outcome. Furthermore, EML4-ALK fusion gene associated with higher protein expression was identified in an embryonal RMS. ALK silencing in RH30 ALK positive cells strongly inhibited invasion capability. Overall, our data suggest a potential role of ALK in pediatric RMS.
URI: http://hdl.handle.net/20.500.12857/115795
DOI: 10.18632/oncotarget.10368
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