Aesthesioneuroblastoma

Abstract

Forty patients were treated or followed up for aesthesioneuroblastoma between 1980 and 1995 at Institut Gustave Roussy, France. There were three T1; seven T2, 15 T3 and 15 T4 lesions. The cervical metastatic rate at presentation was 18 per cent. Distant metastases were detected by bone marrow biopsy and bone scan in three patients at presentation. Treatment modalities included surgery alone in eight patients, radiotherapy alone in three patients, combined modality surgery plus radiotherapy in 11 patients, chemotherapy alone in two patients, chemotherapy plus radiotherapy in 10 patients, and multimodality therapy chemotherapy plus surgery plus radiotherapy in six patients.The five-year survival rate was 51 per cent. Multimodality treatment offered better survival (63 per cent at five years) and disease-free interval (54 months). Overall local, regional, and distant failure rates were 58 per cent, 15 per cent and 40 per cent respectively. Distant metastases commonly occurred in bone (82 per cent). Cervical metastasis was an unfavourable prognostic indicator (0 per cent survival at two years).In conclusion, aesthesioneuroblastoma is sensitive to chemotherapy and radiotherapy. Multimodality therapy should be used initially.

Esthesioneuroblastoma: a meta-analysis and review

“…In the studies with survival data according to the N stage, 2,8,60,61,64,69,78 only 29% of nodepositive patients were treated successfully, compared with 64% of N0 patients, a significant difference (odds ratio 5.1 [95% CI 1.6-17.0]).…”

“…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.…”

“…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]…”

“…Although this classification system has gained popularity, [4][5][6] various attempts have been made to modify the Kadish system for ENB. 7,8 Within the past decade, there has been progress in identifying the origin of ENB, which should lead to more precise diagnosis. In addition, although surgery and radiotherapy have remained the main treatments, chemotherapy has been increasingly used.…”

“…2 The commonest symptoms are a unilateral nasal obstruction (70%), followed by epistaxis (46%). 2,8 However, these symptoms occur in common nasal diseases such as chronic rhinosinusitis or allergic polypoid sinus disease. The key to early diagnosis of ENB is early referral for an intranasal biopsy.…”