Olfactory Neuroblastoma ‐ Management of a rare tumour at the Queensland Radium Institute and literature review

Abstract

Olfactory neuroblastoma (esthesioneuroblastoma) is an extremely rare tumour arising from the olfactory epithelium of the nasal cavity close to the cribriform plate. Most institutions will have little experience in recognising the clinical and histological features, or management of this tumour and reliance is placed on researching the literature when the individual patient presents. This study reviews seven patients with olfactory neuroblastoma treated at the Queensland Radium Institute from 1971 to January 1990. The overall local control rate in this series is 57% (four of seven patients) and 43% of patients (three of seven) remain alive. Conservative surgery and post-operative radiotherapy is recommended for early disease and more radical surgery with post-operative radiotherapy for advanced disease.

Esthesioneuroblastoma: The Princess Margaret Hospital experience

“…Our 5‐ and 10‐year survival rates of 87.9% and 69.2%, respectively, compare favorably with the literature. In a meta‐analysis3 of 25 studies of 390 of patients the reported overall survival at 5 years was 45%, with extremes of 86%28, 29 and 0% 29. However, the authors noted that for the larger studies the survival rates approximated 70%.…”

Esthesioneuroblastoma: a meta-analysis and review

“…Overall survival at 5 years could be extracted from 25 studies; the mean was 45% (SD 22), with extremes of 86% 58 and zero. 57 Disease-free survival at 2 years was found in 17 studies, 2,43,[54][55][56][57][58]60,62,65,66,[68][69][70][71]73,78 that at 3 years in 20 reports 4,6,8,43,[54][55][56][57][58]60,62,65,66,[68][69][70][71][72][73]78 and that at 5 years in 24 publications. 2,4,6,8,43,[54][55][56][57][58][60][61]…”

“…The 26 studies 2,[4][5][6]8,43,[54][55][56][57][58][60][61][62][63][64][65][66][68][69][70][71][72][73]77,78 reported on 390 patients with ENB. Overall survival at 5 years could be extracted from 25 studies; the mean was 45% (SD 22), with extremes of 86% 58 and zero.…”

“…2,6,[54][55][56][57][58][61][62][63][64][65][66][68][69][70][71][72][73]77,78 The distribution was 12% stage A, 27% stage B, and 61% stage C. Mean survival for these three groups was 72% (SD 41), 59% (SD 44), and 47% (SD 16), respectively. Four studies 2,4-6 used the Dulguerov and Calcaterra staging with the following distribution: 25% T1, 25% T2, 33% T3, and 17% T4.…”

Esthesioneuroblastoma: case report

“…In the literature the 5-year recurrence free survival is report to be between 52% and 90% 16,29,40 . ENB recurs locally in up to 60% of patients who undergo surgery 15 , and its locally aggressive behavior is the most common cause of death 44 . Median survival after recurrences was only 12 months 45,46 .…”