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Ethesioneuroblastoma

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Ethesioneuroblastoma
Gruber G, Laedrach K, Baumert B, et al.
Int J Radiat Oncol Biol Phys. 2002;54:486-491.


The purpose of this study was to evaluate long-term outcomes in patients with esthesioneuroblastoma treated with neoadjuvant or definitive radiotherapy (RT).

The study included 28 patients with histologically confirmed esthesioneuroblastoma who, between 1980 and 2001, underwent RT (median dose, 60 Gy [range, 38 to 73 Gy]). The median age of the patients was 58 years (range, 16 to 85 years). According to Kadish classification, 4 patients had stage A disease, 8 patients had stage B disease, and 16 patients had stage C disease. Radical resection was performed in 9 patients before RT and in 4 patients after RT because they were determined to have either stable or progressive disease.

After a mean follow-up of 68 months, 54% of patients were free of tumor progression. The 5- and 10-year local progression-free survival rate was 81% and 51%, respectively, and the disease-free survival rate was 70% and 25%, respectively. Four of 10 deaths were intercurrent, resulting in a cause-specific survival of 77% and 69% at 5 and 10 years, respectively. Radical resection contributed to significantly better local progression-free and disease-free survival (P < .02). Skull base penetration (P < .04), intraorbital extension (P < .04), and Kadish stage C status (P < .06) were important factors contributing to impaired disease-free survival.

Despite doses of up to 73 Gy, radical RT cannot replace radical resection. This suggests that esthesioneuroblastoma is radioresistant. Because of its biology and the high rate of late recurrence, we recommend a radical strategy with resection, high-dose RT, and simultaneous chemotherapy. Further, we must acknowledge that some tumors qualify for palliative treatment only.

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