Intrathoracic malignant peripheral nerve sheath tumor with angiosarcoma in a child with NF1

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in children and adolescents but occur more frequently in NF1 patients. Angiosarcomatous differentiation in MPNSTs is a rare entity with poor prognosis. We report on a 13-year-old boy with intrathoracic angiosarcoma arising in MPNST associated with NF1.