Esthesio Neuroblastomas

Esthesio-neuroblastoma, or olfactory neuroblastoma, is a rare tumor of neural crest origin that arises from olfactory epithelium (117). Having a peak incidence in the third decade of life and occurring most commonly in males, esthesioblastomas usually begin high in the nasal cavity, extend into the paranasal sinuses, and eventually erode intracranially via the cribriform plate. Transgression of dura, brain invasion, intraorbital extension, and cavernous sinus infiltration are potential sequelae of intracranial disease. Metastatic dissemination, usually to regional lymph nodes, lungs, or bones, occurs in up to 30% of patients at some time during their lifetime. Nasal obstruction, epistaxis, ocular symptoms, and headache are the most common presenting features. More than half of all tumors are of advanced stage at the time of diagnosis. The optimal treatment for this tumor is unsettled (117-119). When intracranial extension is demonstrated, a common, but not universally endorsed treatment protocol includes preoperative radiation, preoperative and postoperative chemotherapy, and craniofacial resection. At the University of Virginia, this strategy provided 5- and 10-yr survival rates of 81% and 55%, respectively (120). Local recurrence is common, which, in some instances, may occur a decade or more after initial therapy.

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