Esthesioneuroblastoma treatment
Primary treatment is controversial. Some institutions believe in upfront combined radiation therapy and chemotherapy prior to craniofacial resection. However, most practice upfront surgery, which classically consisted of endoscopic resection with negative margins for Kadish A and B lesions, and craniofacial resection (bifrontal craniotomy with associated lateral rhinotomy) for Kadish C and D lesions. However, with the advent of endoscopic techniques, the lateral rhinotomy is often replaced with a purely endoscopic approach, unless there is inferior lateral orbital or maxillary involvement, in which case the lateral rhinotomy is frequently used. Finally, some institutions are now managing Kadish stages purely endoscopically, unless they are unable to get negative margins at the time of surgery, in which case conversion to an open approach is performed or SRS is performed; however, this is controversial.
Since 2000s, evolution of the endoscopic endonasal approaches (EEA) has offered the option of minimally invasive techniques in the management of esthesioneuroblastomas (ENB), either as endoscope assisted cranial resection or as pure endoscopic procedures.
There is growing evidence supporting the feasibility of safe and effective resection of esthesioneuroblastoma via an expanded endonasal approach 1).
Endoscopic resection with transnasal craniectomy and subpial dissection (ERTC-SD) can provide good local control, satisfactory survival, and limited morbidity 2).
Pure EEA offer excellent results in the management of ENB. Neoadjuvant radiation treatment is promising although more studies need to establish its role 3).
Intensity modulated radiotherapy (IMRT) and carbon ion radiotherapy (CIRT) are advanced radiation techniques that might improve local tumor control.
Results demonstrate that IMRT, CIRT, a combined approach of IMRT and CIRT as well as reirradiation with CIRT seem to be feasible and effective treatment methods in ENB. 4).
ENB is safely and effectively treated with craniofacial resection (CFR) followed by proton beam irradiation. The high incidence of regional metastases warrants strong consideration for elective neck irradiation. Proton beam radiation is associated with lower rates of severe late-radiation toxicity than conventional radiotherapy 5).