Malignant Intracranial Skull Base Tumors

Olfactory Neuroblastoma (Esthesioneuroblastoma) An uncommon neuroectodermal tumor that arises from the olfactory nasal epithelium, high up in the nasal roof within the olfactory cleft (the space between the superior nasal septum and the middle and superior turbinates on the lateral wall of the nose), attached to the cribriform plate. It affects both sexes equally and occurs in all age groups. Presentation is often non-specific, with nasal obstruction, epistaxis and, less commonly, anosmia and visual problems.

Although the diagnosis relies on biopsy and histology, imaging allows assessment of the extent of the tumor. Woodhead and Lloyd (1988) reviewed imaging in a series of 24 patients and concluded that there were no specific features to identify this tumor. However, a tumor within the ethmoid and upper nasal airway, expanding into the orbit and eroding the roof of the fronto-ethmoid complex or cribriform plate unilaterally in a young patient, is likely to be an olfactory neuroblastoma (see Fig. 15.2).

The natural history of this malignancy is both variable and unpredictable. Metastasis to cervical lymph nodes occurs in 10-30% of patients during the course of the disease and systemic metastasis (lung and bone) in 8-46% of cases [10-13]. Although often slow-growing, it must be considered as a highly malignant neoplasm, requiring radical initial treatment.

Treatment normally involves a combination of radical surgery and radiotherapy. Survival rates of 60% at 3 years and 40% at 5 years are

Fig. 15.2. Sagittal MRI of patient with large olfactory neuroblastoma.

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