Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000.
OBJECTIVE
A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines.


METHODS
Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome.


RESULTS
The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3-11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3-19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0-19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(1/2) years.


CONCLUSIONS
The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.
Departments of Otolaryngology-Head & Neck Surgery, Rigshospitalet, University Hospital of Copenhagen, Copenhagen, Denmark. sat@dadlnet.dk
Sune A
SA
Theilgaard
Christian
C
Buchwald
Peter
P
Ingeholm
Susanne
S
Kornum Larsen
Jesper G
JG
Eriksen
Hanne
H
Sand Hansen
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy / Q000009:adverse effects; D003718:Denmark / Q000453:epidemiology; D018572:Disease-Free Survival; D018304:Esthesioneuroblastoma, Olfactory / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D011183:Postoperative Complications / Q000453:epidemiology; D012042:Registries; D012189:Retrospective Studies; D012907:Smoking / Q000453:epidemiology; D015996:Survival Rate