Treatment of Childhood Esthesioneuroblastoma

The use of multimodal therapy optimizes the chances for survival, with more than 70% of children expected to survive 5 or more years after initial diagnosis.[1-5] Neuromeningeal progression is the most common type of treatment failure.[5,6][Level of evidence C1]

Treatment options according to Kadish stage include the following:[7]

The mainstay of treatment is surgery and radiation therapy. However, esthesioneuroblastoma is a chemosensitive neoplasm, and the use of neoadjuvant chemotherapy can facilitate resection.[5,7-9] Endoscopic sinus surgery offers short-term outcomes similar to open craniofacial resection.[10]; [11][Level of evidence C2] Other techniques such as stereotactic radiosurgery and proton-beam therapy (charged-particle radiation therapy) may also play a role in the management of this tumor.[3,12,13]

Routine neck dissection and nodal exploration are not indicated in the absence of clinical or radiological evidence of disease.[14] Management of cervical lymph node metastases has been addressed in a review article.[14]

Reports have indicated promising results with the increased use of resection and neoadjuvant or adjuvant chemotherapy in patients with advanced-stage disease.[2,5,15-17]; [18][Level of evidence C1] Chemotherapy regimens that have been used with efficacy include the following: