Esthesioneuroblastoma (ENB) is a rare tumor arising out of the nasal vault. Symptoms at the time of presentation include: nasal congestion, anosmia, epistaxis, and pain. Because of the nonspecific nature of these symptoms, patients often present with locally advanced disease. ENB is characterized by a tendency toward invasion of local structures including the paranasal sinuses, orbits, anterior cranial fossa dura, cavernous sinus, and frontal lobe. Less than 5% of patients present with nodal metastases; disseminated disease is usually restricted to patients with recurrence. Historically, patients have been staged using the Kadish staging system (A-limited to the nasal cavity, B-involving the paranasal sinuses and C-extending beyond the nasal cavity and paranasal sinuses). More recently, the Tumor-node-metastasis (TNM) staging system of Dulguerov has become popular. Although patients with nodal disease have significant decrease in survival, overall stage at diagnosis does not consistently predict survival. Tumors are graded using the Hyams classification (grade I-well-differentiated tumors through IV-poorly differentiated tumors). Poorly differentiated tumors must be carefully reviewed to rule out other pathologic entities such as sinonasal undifferentiated carcinomas, which have a distinct and more rapidly progressive course. Unlike stage, histologic grade appears to be prognostic for survival. Treatment is based on stage at diagnosis. Early stage tumors may be considered for treatment with surgery or radiation as a single modality. Locally advanced resectable tumors are usually treated with resection followed by radiation therapy. Patients with unresectable or marginally resectable disease may be treated with combined chemotherapy and radiation therapy. ENB is generally a slow-growing tumor with a high 5-year survival (81%). Recurrences usually occur within the first 2 years; however, late recurrences are common, and hence follow-up must be prolonged. The most common site of recurrence is local (approximately 25%), with regional and distant metastases occurring in 20 and 15% of patients respectively. Salvage for local or regional recurrence is possible in approximately one-third of patients.
- Olfactory neuroblastoma
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