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A Journal on Otorhinolaryngology, Head and Neck Surgery,
Plastic Reconstructive Surgery, Otoneurosurgery
Indexed/Abstracted in: EMBASE, Scopus
Otorinolaringologia 1999 December;49(4):193-9
Strinati F., Bizzotti C., Longari F., Rizzo S., Frenguelli A.
Università degli Studi - Perugia Azienda Ospedaliera «S. Maria» - Terni Dipartimento delle Specialità Medico-Chirurgiche Sezione di Otorinolaringologia «R» (Direttore: Prof. A. Frenguelli)
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Olfactory esthesioneuroblastoma is an uncommon neuroectodermal tumor of the upper nasal cavity, microscopic features of which are not always homogeneous. There are less than 500 cases reported in the literature. This entity represents 1 to 5% of all malignant tumors of the nasal cavity. It was first described by Berger in 1924. The early clinical symptoms are often aspecific and histological diagnosis may be difficult to make from the routine examinations of biopsy specimen. This two facts lead frequently to a delayed diagnosis, so that the prognosis of olfactory neuroblastoma is generally poor. Actually by immunohistochemical findings, it is possible to make an exact diagnosis. A lot of many clinical and histological classifications were proposed in the past, but none is generally accepted. This neoplasia grows slowly and can spread into the adjacent structures like sinuses, orbit and anterior cranial fossa. Locoregional metastases are not frequent and local recurrences are possible over many years. Radical surgery (craniofacial resection) in combination of radiation is the only possible therapy, in order to avoid local recurrences and distant metastasis. Two cases of olfactory esthesioneuroblastoma are described and the main characteristics of this tumor reported in the literature are reviewed. The description of symptoms, microscopic and immunoistochemical findings, imaging technique and the best therapy to carry out are also underlined.