Esthesioneuroblastoma: The Hospital of Santa Creu i Sant Pau Experience 2000–2008. A Retrospective Study of 11 Cases

Introduction: Esthesioneuroblastoma is an uncommon malignant tumor of neuroectodermal origin that tends to arise in the nasal vault and anterior cranial base. It is known for its low incidence; consequently, there are few articles in relation to the treatment of this kind of lesion.

Aim: The aim of this study is to review our experience concerning the treatment of esthesioneuroblastomas and to evaluate the role of the subcranial approach (craniofacial resection) combined with a transnasal endoscopic access in the final results of our patients.

Method: A retrospective study was conducted of 11 cases of esthesioneuroblastomas that were treated in our hospital from 2000 to 2008. There were 3 women and 8 men, with a mean age of 42 years (range, 20–71 years). Presenting symptoms were nasal obstruction (81%), epistaxis (27%), visual loss (18%), headache (18%), and others. Using the Kadish classification, 3 were stage B and 8 were stage C; according to the Dulgerov classification, 3 were T2, 4 were T3, and 4 were T4. A subcranial approach was used in 73%(n = 8) with verification of complete resection using a transnasal endoscopic access. A subtotal resection was achieved in 1 patient with a transnasal endoscopic approach. The other 2 cases were considered unresectable; a biopsy was made by endoscopic approach. The mean follow-up was 44.4 months (range, 12–84 months).

Results: A complete resection was achieved in all patients who underwent a subcranial approach. Of these 8 cases, 62%(n = 5) were alive without disease, 12.5%(n = 1) was alive with disease, and 25%(n = 2) died of their disease. The patient who underwent a subtotal endoscopic resection was alive without disease and the other 2 cases of biopsy with chemotherapy and radiotherapy were alive with controlled disease. Postoperative complications occurred in 36%(n = 4). The most common complications were osteomyelitis of the bone flap (n = 2), wound infection (n = 1), and CSF fistula (n = 1). Radiotherapy was used in all patients, and chemotherapy in 3 cases.

Conclusion: The multidisciplinary treatment of esthesioneuroblastomas is essential to achieve good clinical results for the patients. A subcranial approach, combined with a transnasal endoscopic access, is an effective and safe treatment for the resection of this kind of anterior cranial base tumors.