Abstract
Objectives Evaluation of the changing trends in esthesioneuroblastoma in an Irish context and
review of management options nationally to clarify the best current therapeutic approach
by comparing with international research on this uncommon malignancy.
Design Retrospective review.
Setting Tertiary referral center.
Participants All patients presenting with esthesioneuroblastoma in Beaumont hospital or on the
National Cancer Registry of Ireland between 1994 and 2013.
Main Outcome Measures Recurrence-free and overall survival.
Results During the study period, 32 cases of esthesioneuroblastoma were diagnosed (0.4 per
million per year). Average age at diagnosis was 57 years; however, two cases were
under 20. The majority (62.5%) were male. Patients predominantly presented with epistaxis
or nasal congestion (73%), while two cases were identified incidentally on radiological
investigations. Twenty-seven cases underwent primary surgical management (two post
neo-adjuvant treatment) with seventeen requiring bifrontal craniotomy. Twenty-four
of these received postoperative radiation therapy. Overall, 5-year survival was 65%.
Kadish A/B patients exhibited 100% 5-year disease-specific survival versus 54% in
Kadish C/D (p = 0.011). Hyams grade I/II patients exhibited 75% 5-year disease-specific survival
versus 63% in Hyams grade III/IV (p = 0.005). Patients treated endoscopically exhibited 100% 5-year disease-specific
survival versus 51% in those treated via an open approach (p = 0.102).
Conclusions Many controversies exist in the diagnosis and management of this condition. Despite
this, results from Irish data are mostly concordant with the international literature.
The rising incidence of this disease may represent improved pathological recognition.
An increasing number of esthesioneuroblastoma cases are being successfully treated
via endoscopic surgery.
Keywords
esthesioneuroblastoma - sinonasal malignancy - olfactory neuroblastoma - skull base
