Craniofacial surgery for esthesioneuroblastoma: report of an international collaborative study

Abstract

Introduction Impact of treatment and prognostic indicators of outcome are relatively ill-defined in esthesioneuroblastomas (ENB) because of the rarity of these tumors. This study was undertaken to assess the impact of craniofacial resection (CFR) on outcome of ENB. Patients and Methods Data on 151 patients who underwent CFR for ENB were collected from 17 institutions that participated in an international collaborative study. Patient, tumor, treatment, and outcome data were collected by questionnaires and variables were analyzed for prognostic impact on overall, disease-specific and recurrence-free survival. The majority of tumors were staged Kadish stage C (116 or 77%). Overall, 90 patients (60%) had received treatment before CFR, radiation therapy in 51 (34%), and chemotherapy in 23 (15%). The margins of surgical resection were reported positive in 23 (15%) patients. Adjuvant postoperative radiation therapy was used in 51 (34%) and chemotherapy in 9 (6%) patients. Results Treatment-related complications were reported in 49 (32%) patients. With a median follow-up of 56 months, the 5-year overall, disease-specific, and recurrence-free survival rates were 78, 83, and 64%, respectively. Intracranial extension of the disease and positive surgical margins were independent predictors of worse overall, disease-specific, and recurrence-free survival on multivariate analysis. Conclusion This collaborative study of patients treated at various institutions across the world demonstrates the efficacy of CFR for ENB. Intracranial extension of disease and complete surgical excision were independent prognostic predictors of outcome.

Keywords: adjuvant; combined modality therapy; esthesioneuroblastoma; nose neoplasms/mortality/pathology/surgery/*therapy; olfactory/*therapy; radiotherapy; survival analysis.

Figure 1

With a median follow-up of 56 months, the 5-year overall and disease-specific survival rates calculated by the Kaplan-Meier method were 78% and 83% respectively.

Figure 2

(A) Coronal computed tomography (CT) scan (bone window) demonstrating normal anatomy of the nasal cavity and paranasal sinuses: 1.crista galli 2.cribriform plate 3. lamina papyracea 4.ethmoid sinus. (B) A tumor that is confined to the nasal cavity is staged Kadish A. Coronal sinus CT (bone window) shows a soft tissue mass confined to the right inferior nasal cavity. (C) Involvement of the paranasal sinuses upstages the tumor to Kadish B. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows tumor extension into the right medial maxillary sinus (arrow). (D) Kadish C tumors extend beyond the anatomic confines of the nasal cavity and paranasal sinuses into the orbit or cranial cavity. Coronal T1-weighted post-contrast MRI scan shows intracranial extension of tumor (arrow) with an associated peripheral cyst.

Figure 3

Subtle bone erosion that may be missed on magnetic resonance imaging (MRI) is readily detected on computed tomography (CT) scan. (A) Coronal T1-weighted postcontrast MRI scan shows soft tissue tumor in the nasal cavity and ethmoid sinus, right more than left with questionable right lamina papyracea involvement; (B) Coronal CT scan (bone window) demonstrates definite erosion of the right lamina papyracea (arrow).

Figure 4

Coronal computed tomography scan of the sinuses (bone window) shows intratumoral calcification in an esthesioneuroblastoma of the right nasal cavity.

Figure 5

Differentiating tumor from postobstructive inflammatory changes in the paranasal sinuses may be difficult on computed tomography scan (A), but is generally easy on magnetic resonance imaging (MRI), particularly on the T2-weighted sequence. Coronal T2-weighted MRI scan (B) shows partial extension into the right ethmoid sinus: the tumor is grayish in appearance (closed arrow) while the postobstructive inflammatory change is bright (open arrow).

Figure 6

Magnetic resonance imaging is helpful in delineating relationship of the tumor to the contents of the orbit: (A) Axial precontrast T1-weighted image clearly shows gray tumor in the nasal cavity and ethmoid sinus (closed arrow) and normal orbital fat that appears white (open arrow) indicating no intraorbital extension of tumor. (B) Axial precontrast T1-weighted image shows bilateral orbital involvement by gray tumor. The tumor obliterates the normal fat plane medial to the medial rectus muscles indicating intraorbital extension. This finding is subtle in the right posterior orbit (open arrow) while tumor extension into the left orbit is more obvious (closed arrow).

Figure 7

Coronal T1-weighted post-contrast magnetic resonance imaging scan shows obvious intracranial extension of tumor with peripheral cysts (closed arrow) as well as dural enhancement (open arrow).