Primitive myxoid mesenchymal tumor of infancy is a rare, locally
aggressive, chemotherapy-refractory pediatric sarcoma with a high
morbidity and mortality rate. Surgical resection is the gold standard of
treatment, but a significant number of cases are initially unresectable
due to tumor location. For these individuals, traditional first-line
chemotherapy with vincristine, actinomycin, and cyclophosphamide has
yielded no benefit. Alternatively, therapy with doxorubicin and
ifosphamide-based regimens have shown success in several cases. We
present a case of a pediatric patient with unresectable PMMTI
successfully cured after receiving a combination of ifosphamide and
doxorubicin followed by surgery and adjunctive proton beam radiation.