Management of orbital invasion in esthesioneuroblastoma: 14 years' experience

Abstract

Background: 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: This 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.

Results: There 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.

Conclusions: Our 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.

Keywords: Esthesioneuroblastoma; Orbital invasion; Orbital preservation; Prognostic factors; Radiotherapy.

Fig. 1

Survival curves among various treatment modalities. a Overall survival; b Locoregional relapse free survival

Fig. 2

Comparison of MRI before and after treatment for a 50-year-old patient with ENB (stage of C) who received definitive radiotherapy without surgery. (A) and (B) refer to axial MRI, (C) and (D) refer to coronal MRI. 1, 2 refer to MRI before treatment, after radiotherapy, respectively. MRI before treatment revealed a large mass on the left nasal cavity and ethmoid sinus, which invaded the extraocular muscle of the left orbit (A1 arrowhead, B1 arrowhead). The tumor extended to beyond the midline, left frontal sinus and sphenoid sinus, and frontal lobe (C1 arrowhead, D1 arrowhead). After radiotherapy, the patient had a complete response

Fig. 3

Comparison of MRI before and after treatment for a 38-year-old patient with ENB (stage of C) who received surgery plus postoperative radiation. (A) and (B) refer to axial MRI, and (C) and (D) refer to coronal MRI. 1, 2, and 3 refer to MRI before treatment, after surgery, and after radiotherapy, respectively. MRI before treatment revealed a large mass on bilateral nasal cavity and ethmoid sinus, which invaded the extraconal fat of the right orbit (A1 arrowhead, B1 arrowhead). The tumor extended to right maxillary sinus and sphenoid sinus, bilateral anterior skull base and frontal lobe (C1 arrowhead, D1 arrowhead). After surgery, residual tumor was observed at the site of ethmoid sinus (A2 arrowhead, B2 arrowhead), bilateral anterior skull base and frontal lobe (C2 arrowhead, D2 arrowhead). After postoperative radiation, the residual tumor disappeared

Fig. 4

Survival curves for 52 patients treated with prophylactic elective neck irradiation (PENI)/non-PENI. a Overall survival; b Progression free survival; c Locoregional relapse free survival; d Distant metastasis free survival

Fig. 5

Comparison of MRI before and after treatment for a 43-year-old patient with ENB (stage of C) who received preoperative radiation plus surgery. (A) and (B) refer to axial MRI, and (C) and (D) refer to coronal MRI. 1, 2, and 3 refer to MRI before treatment, after preoperative radiation, and after surgery, respectively. MRI before treatment revealed a large mass on left nasal cavity and ethmoid sinus, which invaded the extraocular muscle of the left orbit (A1 arrowhead, B1 arrowhead). The tumor extended to left anterior skull base (C1 arrowhead, D1 arrowhead)

Fig. 6

Kaplan-Meier OS (left) and PFS (right) curves for patients with ENB stratified by various clinical factors. a Survival curves for patients stratified by Foote stage; b for patients stratified by orbital invasion