Introduction
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a locally
aggressive, treatment refractory soft-tissue sarcoma initially reported
in 20061. Treatment is primarily surgical with more
than two-thirds of the approximately 30 reported cases having undergone
surgery alone. For those individuals in which a complete surgical
resection is not feasible up front, morbidity is high, and event free
survival is poor.2 Here we present the case of an
infant with an unresectable PMMTI who is in remission and off therapy
greater than two years following a combination of systemic chemotherapy,
surgery and adjunctive proton beam radiation. Taken in conjunction with
the eight previously reported cases for which systemic therapy was
attempted, we propose an initial treatment strategy for patients with
initially unresectable PMMTI.