Esthesioneuroblastoma

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare malignant tumor that arises from the olfactory neuroepithelium in the upper nasal cavity (the tissue responsible for the sense of smell). This cancer originates in the cribriform plate region at the roof of the nasal cavity and can invade surrounding structures including the paranasal sinuses, orbits (eye sockets), and the anterior cranial fossa (the front part of the skull base containing the brain). It accounts for approximately 3-6% of all nasal cavity and paranasal sinus cancers. The most common presenting symptoms include progressive unilateral nasal obstruction, recurrent nosebleeds (epistaxis), loss of the sense of smell (anosmia), facial pain, and headaches. As the tumor grows, it may cause visual disturbances, excessive tearing (epiphora), or proptosis (bulging of the eye) if it extends into the orbit. In advanced cases, the tumor can invade the brain, and cervical lymph node metastases or distant metastases to the lungs, bones, or liver may occur. The Kadish staging system is commonly used to classify the extent of disease. Treatment typically involves a multimodal approach. Surgical resection, often performed via craniofacial resection or increasingly through endoscopic endonasal approaches, is the primary treatment. Adjuvant radiation therapy is frequently recommended, particularly for higher-stage tumors or when surgical margins are positive. Chemotherapy may be used in advanced or recurrent cases, often employing platinum-based regimens. The overall prognosis varies significantly by stage, with 5-year survival rates ranging from approximately 50-90% depending on the extent of disease at diagnosis. Long-term follow-up is essential due to the propensity for late recurrences, sometimes occurring more than 10 years after initial treatment.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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