Successful Treatment of Genetically Profiled Pediatric Extranodal NK/T-Cell Lymphoma Targeting Oncogenic STAT3 Mutation

Pediatr Blood Cancer. 2016 Apr;63(4):727-30. doi: 10.1002/pbc.25854. Epub 2016 Jan 5.

Abstract

Extranodal natural killer (NK)/T-cell lymphoma (ENKTCL) is a distinct type of non-Hodgkin lymphoma predominantly observed in Asian and Latin American adult males. A 12-year-old Hispanic female diagnosed with ENKTCL was enrolled in our genomic profiling research protocol. We identified specific somatic alterations consistent with diagnosis of ENKTCL as well as oncogenic mutations in MAP2K1 and STAT3. To our knowledge, this is the first report of an immunophenotypically confirmed and genetically profiled case of ENKTCL in a female pediatric patient in the United States, including its unique treatment and favorable outcome.

Keywords: STAT3; extranodal NK/T-cell lymphoma; genome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Asparaginase / administration & dosage
  • Chemoradiotherapy / methods*
  • Child
  • Cytarabine / administration & dosage
  • DNA Mutational Analysis
  • Dexamethasone / administration & dosage
  • Etoposide / administration & dosage
  • Female
  • Humans
  • Hydrocortisone / administration & dosage
  • Hydroxamic Acids / administration & dosage
  • Ifosfamide / administration & dosage
  • Lymphoma, Extranodal NK-T-Cell / genetics*
  • Lymphoma, Extranodal NK-T-Cell / therapy*
  • MAP Kinase Kinase 1 / genetics
  • Methotrexate / administration & dosage
  • Mutation
  • Positive Regulatory Domain I-Binding Factor 1
  • Precision Medicine / methods*
  • Repressor Proteins / genetics
  • STAT3 Transcription Factor / genetics*
  • Vorinostat

Substances

  • Hydroxamic Acids
  • Repressor Proteins
  • STAT3 Transcription Factor
  • STAT3 protein, human
  • Cytarabine
  • PRDM1 protein, human
  • Vorinostat
  • Etoposide
  • Dexamethasone
  • Positive Regulatory Domain I-Binding Factor 1
  • MAP Kinase Kinase 1
  • MAP2K1 protein, human
  • Asparaginase
  • Ifosfamide
  • Hydrocortisone
  • Methotrexate