%0 Journal Article
%T Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees
%A Gianni Bisogno
%A Pietro Soloni
%A Massimo Conte
%A Marta Podda
%A Andrea Ferrari
%A Alberto Garaventa
%A Roberto Luksch
%A Giovanni Cecchetto
%J BMC Cancer
%D 2012
%I BioMed Central
%R 10.1186/1471-2407-12-117
%X 9 patients (6 males; age 0.9-18 years, median 9.9) were identified by searching the AIEOP (Italian Association of Pediatric Hematology and Oncology) registry and the national databases of rare tumors, soft tissue sarcomas (STS) and neuroblastomas. The data on the cases included in STS treatment protocols were collected prospectively and histology was centrally reviewed; the data and histology concerning the other children were reviewed for the purpose of this analysis.All tumors occurred in the sinonasal region with bone erosion (7 patients) and intracranial (4) or intraorbital (4) extension. Three patients were in Kadish stage B, and 6 in stage C. Complete tumor resection was very difficult to achieve, but adding chemotherapy and radiotherapy enabled tumor control in 8 patients. Response to chemotherapy was evident in 5/7 evaluable cases. Radiotherapy (48.5-60 Gy) was delivered in all children but one, due to early disease progression. With a median follow-up of 13.4 years (range 9.2-22.9), 7 patients are alive in 1st and one in 2nd complete remission. All surviving patients developed treatment-related sequelae, the most frequent being endocrine dysfunctions (4 patients) and craniofacial growth impairments (4 patients).Our findings confirm that ENB in children has an aggressive presentation, but multimodal therapy can cure most patients. Our results are encouraging but future strategies must optimize treatment in terms of survival and related morbidities.Esthesioneuroblastoma (ENB), or olfactory neuroblastoma, is a rare, aggressive tumor of the sinonasal region originating from olfactory neuroepithelium. Its incidence is approximately 0.4-1/1,000,000 population per year and, though it can occur at any age, its incidence peaks in the second and fifth decades of life [1,2]. No gender predilection has been reported and its etiology is unknown, but an infectious genesis has been suggested because the tumor contains viral particles [3,4].In pediatric age, the estimated
%K Esthesioneuroblastoma
%K Olfactory neuroblastoma
%K Rare tumors
%K Nasal tumors
%K Chemotherapy
%K Radiotherapy
%K Late effects
%K Endocrine disorders
%U http://www.biomedcentral.com/1471-2407/12/117