Masa Retroperitoneal Gigante En El Tumor Periférico De La Vaina Neural Asociado A Neurofibromatosis Tipo 1. Reporte De Un Caso Y Revisión De La Literatura

Abstract

[eng] Malignant peripheral nerve sheath tumors (MPNST) are most frequently in patients with neurofibromatosis type 1 (NF-1), with a rapid growth and 5-years survival rates around 33%. We present the case of a 17 years old male with NF-1 and a giant retroperi- toneal MPNST who undergoes a radical surgical treatment, with early tumor recurrence.