A Combined Surgical Approach for the Treatment of a Sino-Naso-Orbital Glomangiopericytoma

Abstract

Glomangiopericytomas are a rare type of sinonasal tumors with high relapse rates. Endoscopic sinus surgery is the classical technique to remove such tumors. Here we report a case of sinonasal glomangiopericytoma with orbital extension, infrequently described in the literature. A 50 year-old woman was referred to our hospital because of a right paranasal painless swelling, which appeared 9 months ago and was slowly increasing in size since her first visit. A Magnetic Resonance Imaging (MRI) revealed a tumor (18 × 25 × 37 mm) centered on the right ethmoidal cells, with adjacent nasal bone erosion and a right orbital invasion. The tumor was removed with a combined surgical approach (endoscopic and trans-sinusal frontal approach) after pre-surgical embolization. Based on the results of the histopathological examination, the diagnosis of a glomangiopericytoma was confirmed. The patient was discharged after one week, and an uneventful long-term follow-up.

Keywords: ENT surgery; endoscopy; glomangiopericytoma; maxillo-facial surgery.

Figure 1

(A) Preoperative MRI (Time Of Flight TOF sequence in axial view; black asterisk) and CT-scan (B) (axial view; white asterisk) (C) (coronal view; white asterisk), (D) (sagittal view; white asterisk) showing an anterior ethmoidal high vascularized tumor invading the right nasal bone and the medial wall of the right orbit. R = right, P = posterior.

Figure 1

(A) Preoperative MRI (Time Of Flight TOF sequence in axial view; black asterisk) and CT-scan (B) (axial view; white asterisk) (C) (coronal view; white asterisk), (D) (sagittal view; white asterisk) showing an anterior ethmoidal high vascularized tumor invading the right nasal bone and the medial wall of the right orbit. R = right, P = posterior.

Figure 2

Visualization of the embolized mass in white (3D view). R = right.

Figure 3

Intraoperative view of the tumor through a trans-sinusal frontal approach.

Figure 4

Intraoperative view after complete removal of the tumor.

Figure 5

Histological examination of glomangiopericytoma: HE coloration (A) and β-catenin immunostaining (B) (×200 magnification) shows richly vascularized proliferation consisting of uniform spindle cells with hyperchromatic nuclei and poorly delineated eosinophilic cytoplasm, forming clusters and storiform structures.

Figure 6

Postoperative (3 weeks) CT-scan in axial view showing the reconstruction of the medial right orbit wall with a titanium mesh. (red arrow pointing at the titanium mesh). R = right, P = posterior.