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Durable disease control with apatinib, irinotecan and temozolomide in a case of metastatic primitive myxoid mesenchymal tumour of infancy
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  • Sze Hon Yu,
  • Amanda Kan,
  • Ka Fai To,
  • Ying Lee Lam,
  • Lai Sim Yan,
  • Anthony Pak Yin Liu,
  • Dennis Ku
Sze Hon Yu
The University of Hong Kong Li Ka Shing Faculty of Medicine
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Amanda Kan
Hong Kong Children's Hospital
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Ka Fai To
The Chinese University of Hong Kong
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Ying Lee Lam
Queen Mary Hospital
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Lai Sim Yan
Hong Kong Children's Hospital
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Anthony Pak Yin Liu
The University of Hong Kong
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Dennis Ku
Hong Kong Children's Hospital
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Abstract

Primitive myxoid mesenchymal tumour of infancy (PMMTI) a rare and aggressive soft tissue tumour driven by alteration in the BCOR gene. Localised disease may be cured by surgical resection but metastatic disease often displays suboptimal chemotherapy response. Here we report the course of a patient with widely metastatic PMMTI, where durable disease control was achieved with the combination of apatinib, a VEGFR inhibitor, and chemotherapy with irinotecan/temozolomide.