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Esthesioneuroblastoma

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Rare Tumors in Children and Adolescents

Part of the book series: Pediatric Oncology ((PEDIATRICO))

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Abstract

Esthesioneuroblastoma (ENB) is the most frequent cancer of the nasal cavity in children, with an estimated incidence of 0.1 per 100,000 children up to 15 years. It is thought to arise from the olfactory neuroepithelium, and it is morphologically similar to neuroblastoma, but biology is different. ENB seems to be more aggressive in children than in adults and can spread to the paranasal sinuses and into the cranial cavity. Nodal involvement is present in up to 25% of children, but distant metastases are rare. MRI is important to delineate the extent of the disease. MIBG scintigraphy is typically negative. The Kadish staging system is used: (a) tumors confined to the nasal cavity, (b) tumors infiltrate the paranasal cavities, (c) tumors extend beyond the nasal and paranasal cavities, and (d) tumors with metastases. Complete resection is important but often difficult to achieve. Modern radiation techniques such as intensity-modulated radiotherapy or proton therapy should be considered. Sarcoma or neuroblastoma-like chemotherapy regimens have been used with similar results, completing the necessary multidisciplinary approach that has progressively improved the outcome of children with ENB. Long-term sequelae including impaired craniofacial growth, endocrine dysfunctions, and loss of sense of smell are common.

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Authors and Affiliations

  1. Children’s University Hospital Cologne, Cologne, Germany

    Barbara Hero

  2. Haematology Oncology Division, Department of Women’s and Children’s Health, University of Padova, Padova, Italy

    Gianni Bisogno

  3. Klinik für Kinder- und Jugendmedizin, Klinikum Dortmund, University Witten/Herdecke, Dortmund, Germany

    Dominik T. Schneider

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  1. Barbara Hero
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  2. Gianni Bisogno
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  3. Dominik T. Schneider
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Correspondence to Gianni Bisogno .

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Editors and Affiliations

  1. Clinic of Pediatrics, Municipal Hospital Dortmund, University Witten-Herdecke, Dortmund, Germany

    Dominik T. Schneider

  2. Department of Hematology and Oncology, University Children’s Hospital, Tübingen, Baden-Württemberg, Germany

    Ines B. Brecht

  3. Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta and Emory University, Atlanta, GA, USA

    Thomas A. Olson

  4. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Milano, Italy

    Andrea Ferrari

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Hero, B., Bisogno, G., Schneider, D.T. (2022). Esthesioneuroblastoma. In: Schneider, D.T., Brecht, I.B., Olson, T.A., Ferrari, A. (eds) Rare Tumors in Children and Adolescents. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-92071-5_11

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