Esthesioneuroblastoma

KEY CONCEPTS

• Esthesioneuroblastoma (ENB) is an uncommon neoplasm arising from the olfactory epithelium high in the nasal vault, which frequently invades the skull base, orbit, and cranial vault.
• Both the Hyams histologic grading system and Kadish staging stratification have prognostic value with respect to outcomes in patients with ENB.
• Surgery remains the mainstay of treatment with a goal of negative margins. Surgical techniques were traditionally craniofacial approaches, with the addition of endoscopic techniques recently.
• Radiotherapy is commonly used postoperatively. Preoperative radiotherapy has been used with benefit in advanced-stage ENB, and chemotherapy is also often used.
• Radiosurgery has been effectively used for residual or recurrent tumors of limited size.