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Orbital Paraganglioma: A Systematic Review.
Natalie Huang1, Hani M Rayess1, Peter F Svider1
1Department of Otolaryngology - Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States.
Journal of Neurological Surgery. Part B, Skull Base
|July 17, 2018
Summary
Orbital paragangliomas are rare tumors. This systematic review found they typically present with proptosis and have an excellent prognosis, often managed with surgical excision.
Area of Science:
- Ophthalmology
- Neurosurgery
- Oncology
Background:
- Orbital paragangliomas are rare neuroendocrine tumors.
- These tumors arise from paraganglionic cells within or adjacent to the orbit.
- Understanding their characteristics is crucial for effective management.
Purpose of the Study:
- To systematically review the literature on orbital paragangliomas.
- To evaluate clinical presentation, diagnostic methods, management strategies, and patient prognosis.
Main Methods:
- Systematic review adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines.
- Comprehensive literature search across PubMed, Cochrane, Embase, and Web of Science.
- Inclusion of original English-language human studies.
Main Results:
- Twenty-seven articles comprising 28 patients were analyzed.
- Common symptoms include proptosis (89.2%) and visual acuity changes (67.9%).
- Intraconal location was most frequent (92.9%); surgical excision was the primary treatment (42.9%), with a 92.9% survival rate.
Conclusions:
- Orbital paragangliomas are rare but have an excellent prognosis.
- Proptosis is a hallmark presentation.
- Surgical excision is the mainstay, with exenteration or radiation for invasive cases.
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