Tumor maligno de bainha de nervo periférico acometendo o nervo laríngeo recorrente em uma crian?a com neurofibromatose tipo 1

摘要

An 11-year-old girl presented with hoarseness, dysphagia, and swelling in the left side of her neck. She had a previous diagnosis of neurofibromatosis type 1, presenting with multiple café-au-lait spots and subcutaneous neurofibromas (Figure A). Magnetic resonance imaging showed a mass in the left side of her neck (Figure B and C). The final diagnosis was a malignant peripheral nerve sheath tumor (Figure D) arising from the left recurrent laryngeal nerve. A malignant peripheral nerve sheath tumor is an uncommon, aggressive soft tissue sarcoma of neural origin. It is defined as a nerve sheath tumor arising from a peripheral nerve, a pre-existing peripheral nerve sheath tumor, or in the setting of neurofibromatosis type 11,2.