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 1 st 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.
| Original language | English |
|---|---|
| Article number | 117 |
| Journal | BMC Cancer |
| Volume | 12 |
| DOIs | |
| Publication status | Published - Mar 25 2012 |
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Keywords
- Chemotherapy
- Endocrine disorders
- Esthesioneuroblastoma
- Late effects
- Nasal tumors
- Olfactory neuroblastoma
- Radiotherapy
- Rare tumors
ASJC Scopus subject areas
- Oncology
- Cancer Research
- Genetics
Cite this
Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees. / Bisogno, Gianni; Soloni, Pietro; Conte, Massimo; Podda, Marta; Ferrari, Andrea; Garaventa, Alberto; Luksch, Roberto; Cecchetto, Giovanni.
In: BMC Cancer, Vol. 12, 117, 25.03.2012.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees
AU - Bisogno, Gianni
AU - Soloni, Pietro
AU - Conte, Massimo
AU - Podda, Marta
AU - Ferrari, Andrea
AU - Garaventa, Alberto
AU - Luksch, Roberto
AU - Cecchetto, Giovanni
PY - 2012/3/25
Y1 - 2012/3/25
N2 - 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 1 st 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.
AB - 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 1 st 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.
KW - Chemotherapy
KW - Endocrine disorders
KW - Esthesioneuroblastoma
KW - Late effects
KW - Nasal tumors
KW - Olfactory neuroblastoma
KW - Radiotherapy
KW - Rare tumors
UR - http://www.scopus.com/inward/record.url?scp=84858727930&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84858727930&partnerID=8YFLogxK
U2 - 10.1186/1471-2407-12-117
DO - 10.1186/1471-2407-12-117
M3 - Article
C2 - 22443159
AN - SCOPUS:84858727930
VL - 12
JO - BMC Cancer
JF - BMC Cancer
SN - 1471-2407
M1 - 117
ER -