Esthesioneuroblastoma (ENB)

AKA: Olfactory neuroblastoma.

Esthesioneuroblastoma is a rare malignant tumor of sinonasal origin. These tumors typically present with unilateral nasal obstruction and epistaxis, and diagnosis is confirmed on biopsy.


Case series

Nineteen patients from Brescia received endoscopic resection with transnasal craniectomy and subpial dissection (ERTC-SD) and 11 had pathological-proven brain invasion. Histologies were 6 olfactory neuroblastomas (ONB), 3 neuroendocrine carcinomas, and 2 intestinal-type adenocarcinomas. Mean follow-up was 21.9 months. Three-year overall, local recurrence-free, and distance recurrence-free survivals were 65.5%, 81.8%, and 68.2%, respectively. Overall and distant recurrence-free survivals were significantly better in patients with ONB (P = 0.032 and P = 0.013, respectively). Hospitalization ratio was 4.1%. Complication rate was 10.5%.

In selected nasal-ethmoidal tumors with brain invasion, ERTC-SD can provide good local control, satisfactory survival, and limited morbidity 1).

Klironomos et al., presented the use of pure EEA in the management of ENB in the Toronto Western Hospital, along with a literature review. They retrospectively reviewed the clinical, radiology and pathology records of patients with ENB treated during the period July 2006 to January 2016. During the above period, ten patients with ENB were treated using pure EEA. The mean age was 47.5 years. The gender distribution was: eight males, two females. The most common presenting symptoms were nasal obstruction and discharge or epistaxis (8/10). The mean duration of symptoms was 1.5 years. All patients had preoperative confirmation of ENB by biopsy. Five patients received neoadjuvant radiation and four underwent postoperative radiation. One patient did not receive any radiotherapy and no patient received chemotherapy. Gross total resection was achieved in all patients and intraoperative microscopically negative surgical margins achieved in 9/10 (90%). No major intraoperative complications occurred. The most common postoperative complication was nasal infection. Cerebrospinal fluid leak was noted in one patient. During the follow-up period of 6-120 months (mean 74.8) two cases of neck lymph node recurrence were observed. No deaths due to the disease occurred during the follow-up period. Pure EEA offer excellent results in the management of ENB. Neoadjuvant radiation treatment is promising although more studies need to establish its role 2).

There is a scarcity of data about different treatment strategies. Intensity modulated radiotherapy (IMRT) and carbon ion radiotherapy (CIRT) are advanced radiation techniques that might improve local tumor control.

In a retrospective analysis of 17 patients with ENB (Kadish stage ≥ C: 88%; n = 15). Four patients had already undergone previous radiotherapy (RT). The treatment consisted of either IMRT (n = 5), CIRT (n = 4) or a combination of both techniques (n = 8). Median follow-up was 29 months. (3) Results: In patients that had not been irradiated before (n = 13), calculated overall survival (OS) and progression free survival (PFS) rates after 48 months were 100% and 81% respectively (Kaplan-Meier estimates). Two of four patients that underwent reirradiation died after RT, presumably due to tumor progression. Besides common toxicities, five patients (30%) showed mostly asymptomatic radiation-induced brain changes, most likely due to a disturbance of the blood-brain barrier.

The 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. 3).

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 4).

The National Cancer Database (NCDB) was used to identify patients diagnosed with ENB between 2004 to 2015. Patients were excluded based on the ability to properly stage their disease as well as the availability of treatment data.

Eight-hundred eighty-three patients had sufficient data for analysis. On multivariate analysis, age and government insurance were associated with primary surgical treatment, whereas tumor stage, gender, race, hospital type and volume, and comorbidity score were not. Age, charlson-deyo comorbidity (CDCC) score, hospital volume, and nodal status were found to be predictors of survival. Multivariate-analysis controlling for stage failed to demonstrate clear survival differences between staging in both TNM and Kadish systems. T-stage and the presence of regional nodal metastasis were associated with an increased risk of positive margins on multivariate analysis.

Although primary surgical management and positive margins can be predicted by certain patient and tumor factors, clinical staging systems for ENB poorly predict prognosis over a 10-year horizon 5).

Esthesioneuroblastoma case reports

A case of non-contiguous meningeal recurrence of olfactory neuroblastoma presenting as a giant frontal mass. A 66-year-old woman was admitted with a left nasal intranasal localized tumor without cranial extension and gross total removal was achieved. Pathological examination showed olfactory neuroblastoma and radiation therapy was added in a limited region of the removal cavity. Radiological follow-up continued for 10 years and there was no local recurrence. Sixteen years after radiation therapy, the patient found a slight frontal mass gradually growing. Magnetic resonance imaging revealed an enhanced mass lesion of 7 cm in thickness and 9 cm in diameter associated with marked thickness of the frontal bone, intradural cystic mass compressing the bilateral frontal lobe, and no local recurrence. A second operation was performed followed by radiotherapy and we diagnosed no-contiguous meningeal recurrence of metastatic olfactory neuroblastoma. Olfactory neuroblastoma is a locally aggressive tumor. Although metastasis of this tumor has been reported, non-contiguous spread to the dura is rare. Understanding the route of remote metastasis and careful evaluation after primary treatment are needed to avoid misdiagnosis and treatment delays 6).



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.

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.

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.

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.

Joshi RR, Husain Q, Roman BR, Cracchiolo J, Yu Y, Tsai J, Kang J, McBride S, Lee NY, Morris L, Ganly I, Tabar V, Cohen MA. Comparing Kadish, TNM, and the modified Dulguerov staging systems for esthesioneuroblastoma. J Surg Oncol. 2019 Jan;119(1):130-142. doi: 10.1002/jso.25293. Epub 2018 Nov 22. PubMed PMID: 30466166.

Saito A, Sasaki T, Inoue T, Narisawa A, Inoue T, Suzuki S, Ezura M, Uenohara H. Non-contiguous Meningeal Recurrence of Olfactory Neuroblastoma: A Case Report and Literature Review. NMC Case Rep J. 2018 Jun 28;5(3):69-72. doi: 10.2176/ eCollection 2018 Jul. PubMed PMID: 30023143; PubMed Central PMCID: PMC6048349.

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