OBJECTIVETo investigate the clinical feature, prognostic factors and the appropriate treatment modality of esthesioneuroblastoma (ENB).

METHODSThe data of 49 patients with ENB treated from Dec. 1978 to Dec. 2001 were retrospectively reviewed and analyzed. In this series, 3 patients had modified Kadish stage A disease, 15 stage B, 22 stage C, and 9 stage D lesion. The treatment modalities included surgery alone in 4 patients, and radiotherapy alone in 11, surgery plus radiotherapy in 19, radiotherapy plus chemotherapy in 8, surgery plus radiotherapy plus chemotherapy in 7. Statistic analysis was performed using software SPSS 10.0. Overall survival (OS) and disease free survival (DFS) were calculated using Kaplan-Meier method. Differences between survival curves were tested by Log rank method.

RESULTSThe 5-year OS and DFS of the whole group was 60.5% and 41.9%, respectively. The 5-year OS of patients with modified Kadish stage A or B disease and those with stage C or D was 78.4% and 49.7% (chi(2) = 2.10, P = 0.15), and the 5-year DFS was 47.1% and 38.4% (chi(2) = 0.08, P = 0.78), respectively. The 5-year OS of patients with or without neck lymph nodal metastasis was 17.8% and 70.8% (chi(2) = 2.32, P = 0.13), and the 5-year DFS was 0 and 53.4% (chi(2) = 11.67, P < 0.01), respectively. For patients with kfs > or = 80 and those with kfs < 80, the 5-year OS was 69.0% and 30.1% (chi(2) = 7.01, P < 0.01), and 5-year DFS was 46.7% and 24.9% (chi(2) = 6.37, P = 0.01), respectively. As regard to the treatment modality, The 5-year OS was 69.7% for the patients treated with combined modalities and 46.3% for those with surgery alone or radiotherapy alone (chi(2) = 3.49, P = 0.06), and the 5-year DFS were 52.2% and 21.8% (chi(2) = 7.03, P < 0.01), respectively. The 5-year OS was 71.1% for patients who received surgical treatment and 44.6% for those without it (chi(2) = 7.99, P < 0.01), and 5-year DFS was 54.0% and 24.1% (chi(2) = 6.41, P = 0.01), respectively. The 5-year OS and DFS of 11 patients who received radiotherapy alone were 47.7% and 30.7%, respectively. For 19 patients treated by radiotherapy with radical purpose (including patients who received combined modality with radiotherapy plus chemotherapy), the 5-year OS were 33.9% for < 70 Gy patients and 48.0% for > or = 70 Gy (chi(2) = 0.89, P = 0.35), and the 5-year DFS was 13.3% and 33.3% (chi (2) = 4.48, P = 0.03), respectively. For those who received chemotherapy or not, the 5-year OS was 50.0% and 64.9% (chi(2) = 0.91, P = 0.34), and the 5-year DFS was 38.9% and 43.1% (chi(2) = 0.01, P = 0.91), respectively.

CONCLUSIONEsthesioneuroblastoma is more prevalent in the young male adults than female, usually with locally advanced stage lesion when first diagnosed. Performance status and neck lymph node metastasis are significantly correlated with the prognosis. Combined treatment modality consisting of surgery may help to gain more favorable result. Radiotherapy plays an important role in the management of the disease.