Outcome and prognostic factors in olfactory neuroblastoma: a rare cancer network study.
OBJECTIVE
To assess the outcome in patients with olfactory neuroblastoma (ONB).


METHODS
Seventy-seven patients treated for nonmetastatic ONB between 1971 and 2004 were included. According to Kadish classification, there were 11 patients with Stage A, 29 with Stage B, and 37 with Stage C. T-classification included 9 patients with T1, 26 with T2, 16 with T3, 15 with T4a, and 11 with T4b tumors. Sixty-eight patients presented with N0 (88%) disease.


RESULTS
Most of the patients (n = 56, 73%) benefited from surgery (S), and total excision was possible in 44 patients (R0 in 32, R1 in 13, R2 in 11). All but five patients benefited from RT, and chemotherapy was given in 21 (27%). Median follow-up period was 72 months (range, 6-315). The 5-year overall survival (OS), disease-free survival (DFS), locoregional control, and local control were 64%, 57%, 62%, and 70%, respectively. In univariate analyses, favorable factors were Kadish A or B disease, T1-T3 tumors, no nodal involvement, curative surgery, R0/R1 resection, and RT-dose 54 Gy or higher. Multivariate analysis revealed that the best independent factors predicting the outcome were T1-T3, N0, R0/R1 resection, and total RT dose (54 Gy or higher).


CONCLUSIONS
In this multicenter retrospective study, patients with ONB treated with R0 or R1 surgical resection followed by at least 54-Gy postoperative RT had the best outcome. Novel strategies including concomitant chemotherapy and/or higher dose RT should be prospectively investigated in this rare disease for which local failure remains a problem.
Department of Radiation Oncology, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland. mahmut.ozsahin@chuv.ch
Mahmut
M
Ozsahin
Guenther
G
Gruber
Olimpia
O
Olszyk
Omur
O
Karakoyun-Celik
Berrin
B
Pehlivan
David
D
Azria
Martine
M
Roelandts
Johannes H A M
JH
Kaanders
Mustafa
M
Cengiz
Marco
M
Krengli
Oscar
O
Matzinger
Abderrahim
A
Zouhair
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D003131:Combined Modality Therapy / Q000379:methods; D018572:Disease-Free Survival; D018304:Esthesioneuroblastoma, Olfactory / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity / Q000473:pathology; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult