Outcome and Prognostic Factors in Olfactory Neuroblastoma: A Rare Cancer Network Study

• Published in: International journal of radiation oncology, biology, physics Vol. 78; no. 4; pp. 992 - 997
• Main Authors: Ozsahin, Mahmut, M.D., Ph.D, Gruber, Guenther, M.D, Olszyk, Olimpia, M.D, Karakoyun-Celik, Omur, M.D, Pehlivan, Berrin, M.D, Azria, David, M.D., Ph.D, Roelandts, Martine, M.D, Kaanders, Johannes H.A.M., M.D., Ph.D, Cengiz, Mustafa, M.D, Krengli, Marco, M.D, Matzinger, Oscar, M.D, Zouhair, Abderrahim, M.D
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 15-11-2010; Elsevier
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Analysis of Variance; Biological and medical sciences; BODY; Cancer; CHEMOTHERAPY; Combined Modality Therapy - methods; Disease-Free Survival; DISEASES; Esthesioneuroblastoma; Esthesioneuroblastoma, Olfactory - mortality; Esthesioneuroblastoma, Olfactory - pathology; Esthesioneuroblastoma, Olfactory - therapy; FACE; Female; HEAD; Hematology, Oncology and Palliative Medicine; Humans; Male; Medical sciences; MEDICINE; Middle Aged; Nasal Cavity - pathology; Neoplasm Staging; NEOPLASMS; Neurology; NOSE; Nose Neoplasms - mortality; Nose Neoplasms - pathology; Nose Neoplasms - therapy; NUCLEAR MEDICINE; Olfactory neuroblastoma; Otorhinolaryngology. Stomatology; RADIOLOGY; RADIOLOGY AND NUCLEAR MEDICINE; RADIOTHERAPY; Radiotherapy Dosage; RESPIRATORY SYSTEM; Retrospective Studies; SURGERY; THERAPY; Treatment Outcome; Tumors; Tumors of the nervous system. Phacomatoses; Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology; Young Adult; Esthesioneuroblastoma; Chemotherapy; Radiotherapy; Surgery; Olfactory neuroblastoma; Nose disease; Nervous system diseases; Neuroblastoma; Malignant tumor; Treatment; Olfactory pathway; Olfactory nerve; ENT disease; Evolution; Autonomic neuropathy; Cancer
• ISSN: 0360-3016; 1879-355X
• DOI: 10.1016/j.ijrobp.2009.09.019

Purpose To assess the outcome in patients with olfactory neuroblastoma (ONB). Methods and Materials 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). Conclusion 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.