Granular Cell Tumor of the Orbit: Review of the Literature and a Proposed Treatment Modality
| Autor: | Nahyoung Grace Lee, Paula Cortes Barrantes, Paul Zhou, Myrsini Ioakeim-Ioannidou, Shannon M. MacDonald |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male medicine.medical_specialty Neoplasm Residual Soft Tissue Neoplasm genetic structures Proton Beam Radiation Therapy Inferior rectus muscle medicine Humans Granular cell tumor business.industry General Medicine medicine.disease Magnetic Resonance Imaging Symptomatic relief eye diseases Ophthalmology medicine.anatomical_structure Granular Cell Tumor Oculomotor Muscles Optic nerve Orbital Neoplasms Surgery Radiology Differential diagnosis business Orbit Orbit (anatomy) |
| Zdroj: | Ophthalmic Plastic & Reconstructive Surgery. 38:122-131 |
| ISSN: | 0740-9303 |
| Popis: | Purpose To document a unique case of granular cell tumor of the orbit, located lateral to and abutting the optic nerve, that benefited from treatment with proton beam irradiation, with a comprehensive review of the literature on granular cell tumor of the orbit. Methods Clinicopathologic case report with detailed imaging features and histopathologic and immunohistochemical evaluation for cytoplasmic tumor biomarkers differentiating granular cell tumor (GCT) from it mimicking lesions with relevant literature citations. The authors reviewed 20 cases of orbital GCT from 2011 to 2020 in addition to 40 cases from 1948 to 2011 and included a summary of imaging and clinical features, outcomes, and recommended treatment modalities. Results A 32-year-old man with 1-year history of left retrobulbar pain and diplopia on lateral gaze, intermittent left eyelid swelling, and a tonic left pupil was found to have a fusiform intraconal mass extending toward the orbital apex and abutting the optic nerve. Histopathologic and immunohistochemical investigations collectively supplied data diagnostic of a GCT with an initial low proliferation rate. GCT is a soft tissue neoplasm that originates in the nervous system and can occur anywhere in the body. This enhancing tumor is isointense to gray matter on T1-weighted MRI, hypointense on T2. After an incisional biopsy, the patient's symptoms persisted, and follow-up imaging several months later revealed further growth of the mass. The impossibility of complete surgical removal prompted the decision to treat with proton beam radiation therapy, which resulted in substantial regression in the size of the residual tumor. Most frequently involving the inferior rectus muscle (42%), orbital GCT is usually benign with only 4 reported cases of malignant orbital GCT (7%). Wide surgical resection with complete removal is usually curative for benign orbital GCT, and proton beam radiation therapy can aid in tumor shrinkage. Conclusions GCT should be considered in the differential diagnosis when encountering patients with mass lesions involving the extraocular muscles, peripheral nerves, or less frequently, the optic nerve or orbital apex. Immunohistochemical analysis of biopsied tissue is required for the definitive diagnosis of GCT. Consideration of adjuvant therapies such as proton beam radiation therapy may be appropriate in cases of incomplete surgical resection of benign GCT. Proton beam radiation therapy can be an excellent therapeutic option for symptomatic relief and residual tumor size reduction with an acceptable toxicity profile. |
| Databáze: | OpenAIRE |
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