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Updated: Jan 27, 2026
08:18
Three-Dimensional Reconstruction of Orbital Fractures
Published on: May 16, 2025
647
Orbital Paraganglioma.
1State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China.
The Journal of Craniofacial Surgery
|March 22, 2019
Summary
Orbital paragangliomas are rare neuroendocrine tumors. Surgical excision is the primary treatment, but recurrence is possible, necessitating diligent follow-up and potential radiotherapy.
Area of Science:
- Ophthalmology
- Neuro-oncology
- Pathology
Background:
- Paragangliomas, neuroendocrine neoplasms from neural crest cells, rarely occur in the orbit.
- Orbital paragangliomas present as slow-growing masses causing proptosis.
Observation:
- Two cases (4-year-old male, 27-year-old female) with orbital paragangliomas are presented.
- Imaging revealed well-defined, homogeneous masses with characteristic MRI "salt and pepper" appearance due to vascularity.
- Ultrasound showed homogeneous hypoechoic masses with rich blood flow.
Findings:
- Histopathology and immunohistochemistry confirmed orbital paraganglioma diagnosis.
- Complete tumor excision via orbitotomy was performed.
- One patient experienced recurrence, managed with re-excision and radiotherapy, with no recurrence at 13 years.
- The other patient had no recurrence at 14 years.
Implications:
- Orbital paraganglioma is an extremely rare benign tumor.
- The MRI "salt and pepper" sign is a key diagnostic feature.
- Total surgical excision is the preferred treatment.
- Recurrence risk mandates careful monitoring; radiotherapy is effective for recurrent or incompletely excised tumors.
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