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Open Access Research article

Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees

Gianni Bisogno1*, Pietro Soloni1, Massimo Conte2, Marta Podda3, Andrea Ferrari3, Alberto Garaventa2, Roberto Luksch3 and Giovanni Cecchetto4

Author affiliations

1 Hematology/Oncology Division, Department of Pediatrics, University, Hospital of Padova, Padova, Italy

2 Pediatric Hematology/Oncology Division, G. Gaslini Children's Hospital, Genoa, Italy

3 S.C. Pediatria, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

4 Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Padova, Padova, Italy

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Citation and License

BMC Cancer 2012, 12:117  doi:10.1186/1471-2407-12-117

Published: 25 March 2012

Abstract

Background

Esthesioneuroblastoma (ENB) is a rare, aggressive tumor with no established treatment in children. We analyzed a series of pediatric ENB patients with the aim of improving our knowledge of this disease.

Methods

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.

Results

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).

Conclusions

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.

Keywords:
Esthesioneuroblastoma; Olfactory neuroblastoma; Rare tumors; Nasal tumors; Chemotherapy; Radiotherapy; Late effects; Endocrine disorders