Reconstruction of a pediatric malignant peripheral nerve sheath tumor with a free osteocutaneous fibula flap: a case report

Malignant peripheral nerve sheath tumor (MPNST) is an extremely rare and biologically aggressive tumor. Treatment should ensure wide resection with negative margins because of the high recurrence rate and the risk of metastatic spread as well as the limited benefit of chemotherapy in children. Vascularized bone free flaps are considered the gold standard in reconstructing congenital, infectious, trauma or tumor induced significant defects in the mandible. Optimal reconstruction of mandibular defects is controversial. Simplicity of tecnique with minimal donor site morbidity is a important requirement of a well reconstruction. Important factors such as facial aesthetics and chewing should be taken into account in the planning of pediatric head and neck reconstruction. This study aims to present a MPNST in the left mandible and the mandible’s reconstruction with a free vascularized osteocutaneous fibula flap in a 5-year-old patient. There was not any complication in the 3-year follow-up. Mandibular growth and facial symmetry were maintained. Level of evidence: Level V, therapeutic study.