Durable disease control with apatinib, irinotecan and temozolomide in a
case of metastatic primitive myxoid mesenchymal tumour of infancy
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.