Esthesioneuroblastoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Natural History

Clinical presentation is usually secondary to nasal stuffiness and rhinorrhoea or epistaxis. Presentation is often delayed and symptoms may have been present for many months. Patients often present late with larger tumours which can extend into the intracranial compartment (25-30% at diagnosis) and usually result in anosmia.

Prognosis

Most studies have found a correlation between Hyams grade and prognosis [13,17,21,23]. In a meta-analysis that included five studies in which lesions were graded histologically, the mean five-year survival was 56 percent for those with low-grade lesions (Hyams I and II) versus 20 percent for those with high-grade lesions (Hyams III and IV) [23]. However, a SEER study of 281 patients treated from 1973 to 2010 showed that patients with grade I and II tumors had a 10-year overall survival rate of 67 percent and those with high grade tumors (III and IV) had a 10-year overall survival rate of 34 percent. For high-grade tumors, multivariate analysis showed Kadish stage predicted for worse disease-specific survival and radiation independently predicted for improved disease-specific survival (B-Tajudden).

Treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision. Prognosis is significantly affected by presence of distant metastases.

Metastasis	Five-year survival
No distant metastases	60%
Distant metastases	0%
Small localised tumors	85-90%

no distant metastases: 60% 5-year survival 6 distant metastases: 0% 5-year survival Small localised tumours have a high cure rate, up to 85-90%