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Immune Response against ALK in Children with ALK-Positive Anaplastic Large Cell Lymphoma
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ALK in Neuroblastoma: Biological and Therapeutic Implications

Division of Cellular and Molecular Pathology, Department of Pathology, University of Cambridge, Cambridge CB2 0QQ, UK
Author to whom correspondence should be addressed.
Cancers 2018, 10(4), 113;
Received: 21 March 2018 / Revised: 5 April 2018 / Accepted: 6 April 2018 / Published: 10 April 2018
Neuroblastoma (NB) is the most common and deadly solid tumour in children. Despite the development of new treatment options for high-risk NB, over half of patients relapse and five-year survival remains at 40–50%. Therefore, novel treatment strategies aimed at providing long-term disease remission are urgently sought. ALK, encoding the anaplastic lymphoma kinase receptor, is altered by gain-of-function point mutations in around 14% of high-risk NB and represents an ideal therapeutic target given its low or absent expression in healthy tissue postnatally. Small-molecule inhibitors of Anaplastic Lymphoma Kinase (ALK) approved in ALK fusion-positive lung cancer are currently undergoing clinical assessment in patients with ALK-mutant NB. Parallel pre-clinical studies are demonstrating the efficacy of ALK inhibitors against common ALK variants in NB; however, a complex picture of therapeutic resistance is emerging. It is anticipated that long-term use of these compounds will require combinatorial targeting of pathways downstream of ALK, functionally-related ‘bypass’ mechanisms and concomitant oncogenic pathways. View Full-Text
Keywords: neuroblastoma; ALK; kinase inhibitors neuroblastoma; ALK; kinase inhibitors
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Trigg, R.M.; Turner, S.D. ALK in Neuroblastoma: Biological and Therapeutic Implications. Cancers 2018, 10, 113.

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