Surgical Management of Granular Cell Tumor of the Orbit: Case Report and Literature Review

Jahan Abtahi,Aida Ajan,Iman Malakuti

doi:10.2174/1874210601913010033

Abstract

Introduction:Granular Cell Tumors (GCTs) of the orbit are rare-entity soft-tissue tumors, and few reports have been published in the literature. The treatment of the choice is total excision. Early diagnosis prior to surgery is valuable for the distinction of malignant from benign tumor.Case presentation:We report a case of a 55-year-old woman with a solitary slow-growing mass in the right orbit with the involvement of the rectus inferior muscle, and present a review of the recent literature. The lesion had a diameter of 1 cm and was noticed 2 years before the examination. Excisional biopsy confirmed the diagnosis of GCT. The tumor was resected through a retroseptal transconjunctival approach. The final histological examination revealed findings characteristic of GCT, including positive reaction for protein S-100, SOX10, and calcitonin and negative reaction for desmin, myogenin, Smooth Muscle Antigen (SMA), Melan-A, and HMB-45. There were no signs of malignancy in this sample. Disturbance of motility was not noted by the patient after surgery.Conclusion:GCT should be included in the differential diagnosis of intraorbital lesions, particularly those that involve the orbit muscles. A biopsy is recommended before surgical resection, to exclude malignancy and prevent radical resection.

Highlights

Granular Cell Tumors (GCTs) of the orbit are rare-entity soft-tissue tumors, and few reports have been published in the literature
GCT should be included in the differential diagnosis of intraorbital lesions, those that involve the orbit muscles
A biopsy is recommended before surgical resection, to exclude malignancy and prevent radical resection

Summary

Introduction

Granular Cell Tumors (GCTs) of the orbit are rare-entity soft-tissue tumors, and few reports have been published in the literature. Diagnosis prior to surgery is valuable for the distinction of malignant from benign tumor. Granular Cell Tumor (GCT) is mostly a benign soft-tissue tumor with a Schwann cell phenotype that can arise in any site in the body [1]. GCT was described by Abrikossoff in 1926 as a tumor of the tongue with granular cells derived from striated muscle [3]. This tumor was initially called granular cell myoblastoma or myoblastic myoma due to the location.

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Discussion

Conclusion