Treatment

Determining the optimal treatment for olfactory neuroblastoma is complicated somewhat by the variable clinical course that these tumors can adopt. Some demonstrate a very prolonged clinical course with relapses and recurrences over many years, while others grow in a much more aggressive pattern with rapid and massive local, regional, and distant disease recurrence.92 In spite of this unpredictable clinical behavior, the treatment of choice for

Figure 16-21. Esthesioneuroblastoma on MRI showing enhancement and bone destruction.

esthesioneuroblastoma is surgical resection via an anterior craniofacial approach.99 100 Such an approach can be expected to result in 10-year survival for approximately two-thirds of patients.98,99

Radiotherapy and chemotherapy, in a variety of combinations, have also been employed in the treatment of esthesioneuroblastoma.101104 While over one-half of these tumors demonstrate a brief objective clinical response to a number of single-agent chemo-therapeutics, even tumors demonstrating intracranial extension are potential candidates for surgical resection with acceptable morbidity.100101 Therefore, chemotherapy for esthesioneuroblastoma should be considered only in those tumors that demonstrate distant metastasis or tumors that are deemed to be surgically unresectable due to major morbidity or inability to completely encompass them with clear margins.

The role for radiotherapy in the treatment of esthe-sioneuroblastoma is mainly in a postoperative setting where microscopic positive margins are present, or when a local recurrence arises whose resection would involve major surgical morbidity.98 In these clinical situations, the recent introduction and future application of intensity-modulated radiotherapy (IMRT) may allow for the delivery of higher doses of radiation to the anterior cranial base while minimizing the dose received by surrounding structures.105106

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