====== 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 approach]]es (EEA) has offered the option of minimally invasive techniques in the management of [[esthesioneuroblastoma]]s (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 ((Roxbury CR, Ishii M, Gallia GL, Reh DD. Endoscopic Management of Esthesioneuroblastoma. Otolaryngol Clin North Am. 2016 Feb;49(1):153-65. doi: 10.1016/j.otc.2015.09.010. Review. PubMed PMID: 26614835.)). ---- [[Endoscopic]] [[resection]] with [[transnasal]] [[craniectomy]] and [[subpial]] dissection (ERTC-SD) can provide good local control, satisfactory survival, and limited morbidity ((Mattavelli D, Ferrari M, Bolzoni Villaret A, Schreiber A, Rampinelli V, Turri-Zanoni M, Lancini D, Taglietti V, Accorona R, Doglietto F, Battaglia P, Castelnuovo P, Nicolai P. Transnasal endoscopic surgery in selected nasal-ethmoidal cancer with suspected brain invasion: Indications, technique, and outcomes. Head Neck. 2019 Jan 12. doi: 10.1002/hed.25621. [Epub ahead of print] PubMed PMID: 30636181. )). ---- Pure EEA offer excellent results in the management of ENB. Neoadjuvant radiation treatment is promising although more studies need to establish its role ((Klironomos G, Gonen L, Au K, Monteiro E, Mansouri A, Turel MK, Witterick I, Vescan A, Zadeh G, Gentili F. Endoscopic management of Esthesioneuroblastoma: Our experience and review of the literature. J Clin Neurosci. 2018 Dec;58:117-123. doi: 10.1016/j.jocn.2018.09.011. Epub 2018 Oct 16. Review. PubMed PMID: 30340976. )). ---- 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. ((Liermann J, Syed M, Held T, Bernhardt D, Plinkert P, Jungk C, Unterberg A, Rieken S, Debus J, Herfarth K, Adeberg S. Advanced Radiation Techniques in the Treatment of Esthesioneuroblastoma: A 7-Year Single-Institution's Clinical Experience. Cancers (Basel). 2018 Nov 20;10(11). pii: E457. doi: 10.3390/cancers10110457. PubMed PMID: 30463343; PubMed Central PMCID: PMC6267306. )). ---- 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 ((Herr MW, Sethi RK, Meier JC, Chambers KJ, Remenschneider A, Chan A, Curry WT, Barker FG 2nd, Deschler DG, Lin DT. Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy. J Neurol Surg B Skull Base. 2014 Feb;75(1):58-64. doi: 10.1055/s-0033-1356493. Epub 2013 Sep 20. PubMed PMID: 24498591.)). ===== References =====