Anatomy and Diagnosis

Esthesioneuroblastoma or olfactory neuroblastoma is a malignant tumor of the superior nasal vault that arises from the olfactory neuroepithelium and has a bimodal age distribution with peaks in the second and sixth decades.90 These rare tumors can cause vague symptoms that include epistaxis, nasal obstruction, and headache, with expansile and invasive growth leading to malar swelling, dental pain, proptosis, diplopia and increased intracranial pressure.19

While these tumors exert the majority of their morbidity via direct extension and invasion into surrounding structures, they can metastasize to regional lymph nodes, the lungs or bone in as many as 10 to 20 percent of cases.91,92 Endoscopically, a polypoid mass can often be observed in the superior nasal cavity (Figure 16-20) while radiographic imaging (CT/MRI) demonstrates a contrast-enhancing lesion that has both expansile and destructive growth patterns (Figure 16-21).17,93 Esthesioneuroblastomas are composed of small, round cells and may need to be differentiated from melanoma, lymphoma, and sinonasal undifferen-tiated carcinoma (SNUC) on the basis of architecture (formation of neural rosettes) and immunohistochem-ical markers of neural differentiation (neuron-specific enolase; NSE).19 Recently, esthesioneuroblastoma cells have been shown to have a characteristic chromosomal translocation as well as an extensive series of chromosomal gains and losses that may further permit their accurate identification.94,95

A clinical staging system for esthesioneuroblastoma was originally proposed by Kadish, with numerous modifications subsequently proposed.96-98

In the Kadish staging system, group A is composed of tumors confined to the nasal cavity, group B includes tumors that extend to the paranasal sinuses, and group C is composed of tumors that extend beyond the paranasal sinuses (into the orbit, intra-cranial, infratemporal fossa).96

Figure 16-19. A, Neurofibroma. B, Neurofibroma causing fusiform dilatation of nerve. C, Neurofibroma surgical specimen.
Figure 16-20. Esthesioneuroblastoma.

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