Tumor neuroectodérmico de seios paranasais: diagnóstico e tratamento Primitive neuroectodermal tumor of paranasal sinuses: diagnosis and treatment

Primitive neuroectodermal tumours (PNET) are rare and highly aggressive neoplasms found mainly in children and young adults. They are classified in peripheral or central according to their origin. The diagnosis is based on clinical history, computed tomography and magnetic resonance imaging, but the pathological study is the only way to confirm it. Its very important to distinguish the PNET from other small cell round cell tumors - such as lymphoma, Ewings sarcoma and rhabdomyosarcoma - through immunohistochemical specific markers. The treatment includes surgical resection, chemotherapy and irradiation. The prognosis is poor and the survival rate is variable. This article aims to discuss the clinical, imaging and histological features of the primitive neuroectodermal tumors and their treatment.