Esthesioneuroblastoma: The UCLA experience 1970–1990

Abstract

A retrospective review was conducted of all esthesioneuroblastoma cases treated at UCLA Medical Center from 1970 through 1990. Patients were staged according to the staging systems of Kadish, et al., Biller, et al., and a new staging system proposed by the authors. Of 26 patients treated, 74% were alive at 5 years and 60% were alive at 10 years. Combined treatment with surgery and radiation is advocated since a recurrence-free status was achieved in 92% of the patients, compared with 14% for surgery alone and 40% for radiation alone. A craniofacial resection was performed in 7 patients, all of whom have remained disease free. Negative prognostic factors included: age over 50 years at presentation, female sex, tumor recurrence, and metastasis. The proposed new staging system predicted disease-free status better than the other staging systems.

Esthesioneuroblastoma: a meta-analysis and review

“…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]…”

“…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.…”

“…Three other factors contribute to the controversy. 2 First, the tumour shows varying biological activity, ranging from indolent growth, with patients surviving with known tumour for more than 20 years, to a highly aggressive neoplasm, capable of rapid widespread metastasis with survival limited to a few months. Second, ENB is easily confused with other undifferentiated neoplasms of the nasal cavity.…”

“…Recognising the many shortcomings of this staging system, we proposed a classification based on the more familiar TNM system ( Figure 1; Table 1). 2 The staging can be accomplished before treatment and is largely based on computed tomography (CT) and magnetic resonance imaging (MRI), which are quite reliable in assessing the anatomical extent of disease. Therefore, patients given primary or preoperative radiotherapy can be staged as reliably as those who are candidates for surgery.…”