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Italian Journal of Maxillofacial Surgery 2011 April;22(1):65-8

language: English

A rare case of fetal esthesioneuroblastoma

Ramieri V. 1, Basile E. 1, Roberti M. 2, Marucci G. 2, Festa A. 3, Cascone N. 4, Cascone P. 1

1 Department of Maxillo-Facial Surgery, La Sapienza University School of Medicine, Rome, Italy;
2 Department of Pediatric Oncology, La Sapienza University School of Medicine, Rome, Italy;
3 Department of Nuclear Medicine, La Sapienza University School of Medicine, Rome, Italy;
4 Department of Oftalmology, La Sapienza University School of Medicine, Rome, Italy


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Esthesioneuroblastoma (ENB) is a rare tumor arising from nasal cavities deriving likely from the olfactory epithelium. ENB represents from 3% to 6 % of all neoplasms of the nasal cavities and paranasal sinuses. It is quite impossible to establish its real incidence. The disease seems to be equally distributed among gender, it mainly occurs during the fifth and sixth decade tough incidence may present a unimodal or bimodal peak by age, being extremely rare in newborns. Throughout the analysis of this case report the authors underline the importance of a correct prenatal diagnosis and an early surgical treatment. The authors present the case of a newborn with a bulky naso-fronto-orbitary neoplasm. The patient underwent radical surgical treatment and further excision of a preauricolar metastasis combined with postoperativ chemotherapic treatment. Prognostic factors and survival rates can be hardly defined, when dealing with ENB, because of its rarity. Moreover, the earliness of its outcome in our patient represented a further difficulty. The authors adopted a diagnostic and therapeutic protocol according to the International Guidelines.

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