Intraorbital Granular Cell Tumor Ophthalmologic and Radiologic Findings

Abstract

Granular cell tumor is a rare soft tissue neoplasm that commonly affects the head and neck regions. We describe a case of a granular cell tumor of the orbit including its clinical presentation, histopathology, and magnetic resonance imaging findings.

Granular Cell Tumor of the Orbit: Review of the Literature and a Proposed Treatment Modality

“…In the authors' review, all cases-except 2-presented as a well-defined mass or nodule either on CT or MRI. The 2 exceptions were fusiform masses: the current case and one described by de la Vega et al 8 A fusiform shape is comparatively unusual and parallels fusiform orbital schwannomas arising from the ciliary nerves that may extend through the superior orbital fissure.…”

“…The differential diagnosis when encountering a patient with suspected orbital mass must includes benign entities such as cavernous hemangioma, meningioma, and schwannoma. Epithelial malignancies and lymphoid lesions can present with varied imaging patterns, while peripheral nerve sheath tumors, glioma, lacrimal gland adenoid cystic carcinoma, rhabdomyosarcoma, and metastatic disease 8,22 tend to be well defined. Idiopathic orbital inflammation should also be taken into consideration, 23 but this is more diffuse process with irregular borders.…”

“…5,6 Around 70% develop in the head and neck region; the tongue being the most frequent site of involvement. [7][8][9] It has been described to develop in association with small-to medium-sized nerves, including cranial and peripheral nerves. 10…”

Granular Cell Tumor of the Orbit: Review of the Literature and a Proposed Treatment Modality

“…In the authors' review, all cases-except 2-presented as a well-defined mass or nodule either on CT or MRI. The 2 exceptions were fusiform masses: the current case and one described by de la Vega et al 8 A fusiform shape is comparatively unusual and parallels fusiform orbital schwannomas arising from the ciliary nerves that may extend through the superior orbital fissure.…”

“…The differential diagnosis when encountering a patient with suspected orbital mass must includes benign entities such as cavernous hemangioma, meningioma, and schwannoma. Epithelial malignancies and lymphoid lesions can present with varied imaging patterns, while peripheral nerve sheath tumors, glioma, lacrimal gland adenoid cystic carcinoma, rhabdomyosarcoma, and metastatic disease 8,22 tend to be well defined. Idiopathic orbital inflammation should also be taken into consideration, 23 but this is more diffuse process with irregular borders.…”

“…5,6 Around 70% develop in the head and neck region; the tongue being the most frequent site of involvement. [7][8][9] It has been described to develop in association with small-to medium-sized nerves, including cranial and peripheral nerves. 10…”

Orbital granular cell tumor involving the superior rectus muscle: a case report

“…On T1-weighted MRI, the tumor is isointense to gray matter and hypointense on T2. With contrast, there is a slight to strong enhancement ( 9 , 13 ). In this case, MRI showed a clear elliptical mass in the upper rectus muscle walking area, with clear boundaries and regular morphology.…”

“…GCTs are mostly benign, with malignancy accounting for only 0.5%–2% but with a mortality rate of 60%, and some benign GCTs can become malignant ( 3 ). GCTs commonly occur in females and can appear anywhere in the body, with 70% of cases in the head and neck, the tongue being the most common site, and GCTs in the orbit are rare ( 4 – 9 ). Clinical manifestations vary depending on the tumor’s location.…”

Granular Cell Tumor of the Orbit: Review of the Literature and a Proposed Treatment Modality

“…In the authors' review, all cases-except 2-presented as a well-defined mass or nodule either on CT or MRI. The 2 exceptions were fusiform masses: the current case and one described by de la Vega et al 8 A fusiform shape is comparatively unusual and parallels fusiform orbital schwannomas arising from the ciliary nerves that may extend through the superior orbital fissure.…”

“…The differential diagnosis when encountering a patient with suspected orbital mass must includes benign entities such as cavernous hemangioma, meningioma, and schwannoma. Epithelial malignancies and lymphoid lesions can present with varied imaging patterns, while peripheral nerve sheath tumors, glioma, lacrimal gland adenoid cystic carcinoma, rhabdomyosarcoma, and metastatic disease 8,22 tend to be well defined. Idiopathic orbital inflammation should also be taken into consideration, 23 but this is more diffuse process with irregular borders.…”

“…5,6 Around 70% develop in the head and neck region; the tongue being the most frequent site of involvement. [7][8][9] It has been described to develop in association with small-to medium-sized nerves, including cranial and peripheral nerves. 10…”

Orbital granular cell tumor involving the superior rectus muscle: a case report

“…On T1-weighted MRI, the tumor is isointense to gray matter and hypointense on T2. With contrast, there is a slight to strong enhancement ( 9 , 13 ). In this case, MRI showed a clear elliptical mass in the upper rectus muscle walking area, with clear boundaries and regular morphology.…”

“…GCTs are mostly benign, with malignancy accounting for only 0.5%–2% but with a mortality rate of 60%, and some benign GCTs can become malignant ( 3 ). GCTs commonly occur in females and can appear anywhere in the body, with 70% of cases in the head and neck, the tongue being the most common site, and GCTs in the orbit are rare ( 4 – 9 ). Clinical manifestations vary depending on the tumor’s location.…”

Granular Cell Tumor of the Orbit: Review of the Literature and a Proposed Treatment Modality

“…In the authors' review, all cases-except 2-presented as a well-defined mass or nodule either on CT or MRI. The 2 exceptions were fusiform masses: the current case and one described by de la Vega et al 8 A fusiform shape is comparatively unusual and parallels fusiform orbital schwannomas arising from the ciliary nerves that may extend through the superior orbital fissure.…”

“…The differential diagnosis when encountering a patient with suspected orbital mass must includes benign entities such as cavernous hemangioma, meningioma, and schwannoma. Epithelial malignancies and lymphoid lesions can present with varied imaging patterns, while peripheral nerve sheath tumors, glioma, lacrimal gland adenoid cystic carcinoma, rhabdomyosarcoma, and metastatic disease 8,22 tend to be well defined. Idiopathic orbital inflammation should also be taken into consideration, 23 but this is more diffuse process with irregular borders.…”

“…5,6 Around 70% develop in the head and neck region; the tongue being the most frequent site of involvement. [7][8][9] It has been described to develop in association with small-to medium-sized nerves, including cranial and peripheral nerves. 10…”

Orbital granular cell tumor involving the superior rectus muscle: a case report

“…On T1-weighted MRI, the tumor is isointense to gray matter and hypointense on T2. With contrast, there is a slight to strong enhancement ( 9 , 13 ). In this case, MRI showed a clear elliptical mass in the upper rectus muscle walking area, with clear boundaries and regular morphology.…”

“…GCTs are mostly benign, with malignancy accounting for only 0.5%–2% but with a mortality rate of 60%, and some benign GCTs can become malignant ( 3 ). GCTs commonly occur in females and can appear anywhere in the body, with 70% of cases in the head and neck, the tongue being the most common site, and GCTs in the orbit are rare ( 4 – 9 ). Clinical manifestations vary depending on the tumor’s location.…”