[Malignant peripheral nerve sheath tumors: A case report]. / Tumor maligno da bainha dos nervos periféricos do pulmão: A propósito de um caso clínico.

Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments. Its thoracic localization is a very unusual entity. The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis. Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment. This tumor has a bad prognosis because of high local recurrence and metastasis. The clinical case we describe serves as a glimpse for discussion.