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Esthesioneuroblastoma in Children and Adolescent: Experience on 11 Cases With Literature Review

El Kababri, Maria MD; Habrand, Jean L. MD, PhD; Valteau-Couanet, Dominique MD, PhD; Gaspar, Nathalie MD, PhD; Dufour, Christelle MD; Oberlin, Odile MD

Journal of Pediatric Hematology/Oncology: March 2014 - Volume 36 - Issue 2 - p 91–95
doi: 10.1097/MPH.0000000000000095
Original Articles

Esthesioneuroblastoma is a rare tumor of the olfactory epithelium. This report analyzed 11 children and adolescents treated in a single institution between 1982 and 2002. For 9 patients, therapy consisted of an initial course of chemotherapy before surgical resection and postoperative radiotherapy, for 1 patient an initial course of chemotherapy before radiotherapy and for another resection before radiotherapy with no chemotherapy. Response to chemotherapy was assessed in 9 patients of whom 6 achieved a complete or a partial remission. Ten patients are long-term survivors. The 5-year actuarial disease-free survival and overall survival rate was 91% (95% confidence interval, 62%-98%). Our study indicates that esthesioneuroblastoma is sensitive to chemotherapy and supports the role of combined modalities including neoadjuvant chemotherapy, surgery, and radiation therapy.

Departments of *Pediatric and Adolescent Oncology

Radiation Oncology, Institut Gustave Roussy, Villejuif, France

Unit of Pediatric Hematology and Oncology, Children Hospital of Rabat, University of Mohammed V-Souissi, Rabat, Morocco

The authors declare no conflict of interest.

Reprints: Maria El Kababri, MD, Unit of Pediatric Hematology and Oncology, Children Hospital of Rabat, University of Mohammed V-Souissi, Ibn rochd Avenue, Rabat, 6542, Morocco (e-mail: elkababri.maria@gmail.com).

Received October 3, 2012

Accepted November 21, 2013

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