Esthesioneuroblastoma: irradiation alone and surgery alone are not enough.
OBJECTIVE
To evaluate the long-term outcome of patients with esthesioneuroblastoma treated with neoadjuvant or definitive radiotherapy (RT).


METHODS
Between 1980 and 2001, 28 patients with histologically confirmed esthesioneuroblastoma underwent RT, with a median dose of 60 Gy (range 38-73). The median age was 58 years (range 16-85). According to the Kadish classification, 4 patients had Stage A, 8 Stage B, and 16 Stage C tumors. Radical resection was performed in 13 cases, in 9 before RT and in 4 after RT because of stable or progressive disease. The outcome analyses included the median age (58 years), Kadish stage, skull base penetration, intraorbital extension, resection status, and total dose (<or=60 vs. >60 Gy).


RESULTS
After a mean follow-up of 68 months, 54% of patients were free of tumor progression. The 5- and 10-year local progression-free survival rate was 81% and 51%, respectively, and the disease-free survival rate was 70% and 25%, respectively. Four of ten deaths (4/10) were intercurrent, resulting in a cause-specific survival of 77% and 69% at 5 and 10 years, respectively. Radical resection offered significantly better local progression-free survival and disease-free survival (p <0.02). Skull base penetration (p <0.04), intraorbital extension (p <0.04), and Kadish C stage (p <0.06) were important for impaired disease-free survival.


CONCLUSIONS
Despite doses up to 73 Gy, radical RT cannot replace radical resection, which classifies esthesioneuroblastoma as rather radioresistant. Because of its biology and the high rates of late recurrence, we recommend a radical strategy with resection, high-dose RT, and simultaneous chemotherapy. We are aware that some tumors qualify for palliative treatment only.
Gruber
Günther
G
Department of Radiation-Oncology, University of Berne, Inselspital, Bern, Switzerland. guenther.gruber@insel.ch
Laedrach
Kurt
K
Baumert
Brigitta
B
Caversaccio
Marco
M
Raveh
Joram
J
Greiner
Richard
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018450:Disease Progression; D018304:Esthesioneuroblastoma, Olfactory / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D011879:Radiotherapy Dosage; D018714:Radiotherapy, Adjuvant; D015996:Survival Rate