Esthesioneuroblastoma : a general review of the cases published since the discovery of the tumour in 1924

Esthesioneuroblastoma (ENB) arises from the neuroepithelium in the olfactory rim of the nasal cavity. It accounts for about 3% of all intranasal tumours. Reviews since the first description by Berger and Luc in 1924 never reported more than a hundred cases, stressing the rarity of the tumour. However, a thorough literature review revealed a total of 945 reported cases. In our search we found a total of 1,457 cases chronicled in the literature of which perhaps 487 were cited in more than one paper, bringing the total of reported cases to 945. Author cases accounted for 198 and therefore collaborative efforts accounted for 747 cases. Sex distribution was 53.36% male and 46.64% female. Kadish classification was applied to 553 cases revealing 103 (18.29%) class A cases, 182 (32.33%) class B and 278 (49.38%) class C cases. This distribution was generally stable through the decades. Treatment could be classified in 898 cases. It consisted of surgery alone in 25.17% (226 cases), radiotherapy alone in 18.37% (165 cases), combined surgery and radiotherapy in 43.21% (388 cases) and chemotherapy in 13.2% (119 cases), followed in 11 cases (1.22%) by bone marrow transplant. In the reported cases an overall follow up could be evaluated in 477 cases, while in only 234 cases a five-year follow up was done. The outcome was 68.38% alive and disease free, 12.82% alive with disease and 18.80% dead. From these 20.51% had surgery only, 11.11% radiotherapy and 68.38% combined surgery and radiotherapy. The best survival rates were obtained by combined therapy (72.5% vs. 62.5% surgery alone and 53.85% radiotherapy alone). Death rates were highest after radiotherapy alone (30.77% versus 18.75% in combined therapy and 12.50% after surgery alone). In conclusion, ENB is a rare but not exceptional tumour. It is best treated with combined surgery and radiotherapy. Unfortunately early diagnosis is still uncommon and no significant changes to the proportions of Kadish classes at first diagnosis have been noted in recent decades. A greater awareness of the tumour and earlier diagnosis seems the major focus for future research