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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.

INTRATHORACIC MALIGNANT PERIPHERAL NERVE SHEATH TUMOR WITH ANGIOSARCOMA IN A CHILDWITH NF1