Esthesioneuroblastoma treated with non-craniofacial resection surgery followed by combined chemotherapy and radiotherapy: An alternative approach in limited resources.

BACKGROUND: Esthesioneuroblastoma (ENB) is a rare and aggressive malignant tumor arising from olfactory epithelium. Surgical excision in the form of craniofacial surgical resection (CFR) has shown encouraging results. The purpose of the study is to analyze the outcome of this disease when managed by non-craniofacial resection (NCFR) surgery in limited resources.
METHODS: Between October 1998 and January 2004, eight patients with ENB were treated in the Department of Radiotherapy at KGMU, Lucknow. None of these eight patients underwent CFR surgery. All patients received six cycles of vincristine, adriamycin and cyclophosphamide (VAC) based chemotherapy followed by radiotherapy.
RESULTS: All the patients registered during this period had undergone operative procedures in the form of NCFR surgery except two. Complete response was present in five (62.5%) patients and three (37.5%) patients had partial response. Locoregional relapse-free survival at 3 years was 62.5% and median survival time was 38 months. Disease-free survival and overall survival at 3 years was 72.9 and 71.4%, respectively, and median disease-free survival time was 43 months, while mean overall survival time was 40.7 months as median overall survival time was not reached.
CONCLUSION: Patients in developing countries often present with advanced stages and because of non-availability of technical advances and surgical expertise one tends to approach these patients with palliative intent. Most of the patients in our series were of stage C disease (75%) and still our response rate and survival were encouraging despite the fact that surgery was not optimal. This combination chemoradiotherapy schedule can be used outside the protocol setting where resources are limited.