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

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.

Multimodality Treatment of Pediatric Esthesioneuroblastoma

“…There were three times as many females as males in our series. In a pediatric series from France, eight of the 11 patients were female, while another series from Italy had more male patients [8, 9]. This variation is likely the result of small numbers.…”

“…The utility of surgery in patients who have radiological remission following neoadjuvant chemotherapy or irradiation is unknown. Several reports of long term survival without surgery have been published [8, 9, 15]. …”

Pediatric Cancer in the Head and Neck

“…Erosion of the paranasal sinuses is common. In contrast to neuroblastoma in other locations, metaiodobenzylguanidine (MIBG) scans have been shown to be negative in a series of patients with esthesioneuroblastoma [ 67 ].…”

“…Occasional patients may present with Cushing syndrome or hyponatremia due to ectopic ACTH or ADH production [ 6 , 65 , 66 ]. Pediatric esthesioneuroblastoma seems to have a more aggressive presentation than in adults [ 67 ]. There is no evidence of EBV infection [ 68 ].…”

“…The most important predictors of outcome are tumor stage, treatment modality, lymph node status, and age at diagnosis. The prognosis is better in children in comparison to adult patients [ 67 ], with overall 5-year survival rates of 45.6 % in adults and 88.9 % in children [ 61 , 67 ]. Lymph node metastasis, which occurs in approximately 23 % of the cases, has been considered as an important prognostic factor; therefore an elective neck treatment has been recommended [ 83 -85 ].…”