Esthesioneuroblastoma and Neck Metastasis

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the cribriform olfactory epithelium. Its aggressive biologic behavior is characterized by inapparent submucosal spread, local recurrence, atypical distant metastases, and poor long-term prognosis. Historically, the suspicion of neck metastasis has not been associated with ENB. A retrospective review of all ENB patients treated at the University of North Carolina Hospitals since 1972 has revealed an unusually high incidence of cervical metastases (four of four patients). This prompted a review of the ENB surgical literature. All English-literature series published since the widespread use of craniofacial surgery with accruals of eight or more patients were examined for reports of neck metastasis. Although the cumulative cervical metastatic rate reached 27% (55 of 207 patients), most authors failed to recognize the potential risk of cervical metastasis in ENB. Furthermore, in patients suffering from Kadish stage C disease, the cervical metastatic rate climbed to 44% (25 of 57 Kadish stage C patients). This further emphasizes the importance of the neck as a high-risk metastatic site. These findings suggest that cervical metastases may be an important consideration in the evaluation and treatment of patients suffering from ENB. Strategies for evaluation and management of the neck are discussed.