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Successful Crizotinib-Targeted Therapy of Pediatric Unresectable ERC1::ALK Fusion Sarcoma
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  • Megan L. Wood,
  • Julie C. Fanburg-Smith,
  • James M. Brian,
  • Jason White,
  • Jonathan Powell,
  • Andrew Freiberg
Megan L. Wood
Penn State Health Children's Hospital
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Julie C. Fanburg-Smith
Penn State Health Department of Pathology
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James M. Brian
Penn State Health Department of Radiology
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Jason White
Nemours Children's Hospital Delaware
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Jonathan Powell
Nemours Children's Hospital Delaware
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Andrew Freiberg
Penn State Health Children's Hospital

Corresponding Author:[email protected]

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Abstract

Anaplastic lymphoma kinase ( ALK)-fusion sarcomas are rare, part of the emerging theoretically-targetable tyrosine-kinase RAS::MAPK-pathway fusion myopericytic-ovoid-sarcomas. We report our clinicopathologic and treatment experience with an ALK-fusion sarcoma. A novel ERC1-unaligned-ALK-fusion infiltrative nonmetastatic low-grade sarcoma of the right hand of a 15-month-old male was treated with crizotinib, an ALK tyrosine-kinase inhibitor as oral monotherapy, inducing complete radiographic and clinical resolution by 10 months and sustained response now 8 months after elective discontinuation. Crizotinib can successfully be used to treat unresectable novel ALK-fusion sarcomas.