Abstract 964P
Background
Olfactory neuroblastoma (ONB) is a rare neoplasm of sinonasal tract with a peculiar behaviour. Limited prognostic factors are available, consisting in stage, grading (according to Hyams' criteria) and Ki67 value. The project aims at studying the pathways of ONB through gene expression analysis and correlating them with clinical outcome.
Methods
We collected a series of ONB treated with curative intent at the ENT of Spedali Civili, Brescia and Ospedale di Circolo Varese, Italy. Clinical data of the patients were retrieved, as well as histological specimens, whose diagnosis was re-evaluated by two expert pathologists. We performed gene expression profiling on FFPE samples using Affymetrix Clariom S microarray, and carried out functional enrichment analysis to investigate key pathways associated with progression-free survival (PFS).
Results
A series of 42 patients treated between 2001 and 2019 was considered. One patient was excluded due to poor quality of FFPE sample. Main characteristics of the patients: mainly male (52%); median age 53 year; stage I-II 17%, III-IV 83%; Hyams grade I 7%, II 45%, III 48%. Patients were treated by surgery and 79% received postoperative radiation, while only 1 patient received also neoadjuvant chemotherapy. After a median follow up of 51 months, we identified disease progression in 12 cases. Median PFS was 38,8 months (5 - 99). Clinical characteristics (gender, global stage, Hyams grade, T stage and N stage) were not associated with outcome. Patients experiencing recurrence had a disease characterized by enrichment mainly in pathways related to TGF-beta binding, regulation of cytokine biosynthesis, toll-like receptor 4, PIP3, p-53 mediated apoptosis signalling in response to DNA damage, TNF and IFN alfa; on the contrary, patients without any recurrence after surgery showed increased expression of genes related to DNA methylation, ubiquitin ligase complex and retinoid acid binding.
Conclusions
ONB is characterized by heterogeneous gene expression pathways, related to patient’s outcome. Definition of characterizing transcriptomic pathways may pave the way to tailored treatment approaches.
Clinical trial identification
Editorial acknowledgement
Legal entity responsible for the study
The authors.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.