Management of orbital invasion in esthesioneuroblastoma: 14\u2009years\u2019 experience

Ruichen Li,Li Yan,Shengzi Wang,Huatao Quan,Wenjia Zhu,Shu Tian,Yi Zhu

doi:10.1186/s13014-019-1313-1

Abstract

BackgroundThere is a scarcity of data about the prognostic value of orbital invasion in esthesioneuroblastoma (ENB), as well as about its management strategies. Indications for the preservation of orbital contents remain controversial, and the evaluation of orbital invasion has been ill defined.MethodsThis retrospective analysis contained 60 ENB patients with orbital invasion who underwent radiotherapy with or without surgery over the past 14 years. Orbital invasion was classified into three grades.ResultsThere were 52 patients at stage C and 8 at stage D, according to Foote classifications. Grade I, grade II and grade III orbital invasion was detected in 12, 23, and 25 patients, respectively. The median follow-up was 57 months (IQR 32–95 months). Fourteen patients received radical radiotherapy, with a 5-year overall survival (OS) of 63.5%; 46 received surgery plus radiation, with a 5-year OS of 70.7%; and the difference was not statistically significant (p = 0.847). Orbital preservation was feasible in 100% of cases, including 18 cases that extended to extraocular muscles or the eye globe. Five-year locoregional relapse-free survival was 100% in patients with prophylactic elective neck irradiation (PENI) and 58.1% in patients without PENI (p = 0.004). Univariate analysis showed that grade II/III orbital invasion was associated with poorer OS and progression-free survival. Neck metastasis (with a Foote stage of D) was independently associated with shorter OS and distant metastasis–free survival in multivariate analysis.ConclusionsOur data suggested that primary radiotherapy achieved comparable survival to surgery plus radiotherapy in advanced ENB. Invasion of either the extraocular muscles or the eye globe is not a contraindication for eye-sparing surgery. Orbital invasion in grade II/III was significantly associated with adverse survival outcomes. Prophylactic radiotherapy to the neck with N0 significantly reduces the risk of regional recurrence.

Highlights

Esthesioneuroblastoma (ENB) is a rare malignant tumor of the nasal vault that is usually diagnosed at a locally advanced stage [1]
Clinical features A total of 60 patients were included in this study, which consisted of 44 males (73.3%) and 16 females (26.7%), with a median age of 54 years
Organ preservation and adverse reactions of normal tissues We performed surgical resection with orbital preservation in 46 patients (100%), including 18 cases that extended to extraocular muscles or the eye globe, and no patient underwent orbital exenteration

Summary

Introduction

Esthesioneuroblastoma (ENB) is a rare malignant tumor of the nasal vault that is usually diagnosed at a locally advanced stage [1]. Orbital invasion is frequently observed in ENB involving the ethmoid sinus. Whether the prognostic value of the degree of orbital invasion is applicable to ENB remains an open question due to the rarity of ENB. The periorbit plays an important role against invasion and is recognized as a “landmark” of orbital exenteration over time in sinonasal tumors [5,6,7]. There is a scarcity of data about the prognostic value of orbital invasion in esthesioneuroblastoma (ENB), as well as about its management strategies. Indications for the preservation of orbital contents remain controversial, and the evaluation of orbital invasion has been ill defined

Methods

Results

Discussion

Conclusion