Orbital Paraganglioma: A Systematic Review

Natalie Huang; Hani M. Rayess; Peter F. Svider; Nadim M. Rayess; Adam Folbe; Paul D. Langer; Jean-Anderson Eloy; Michael Carron

doi:10.1055/s-0037-1615750

Journal of Neurological Surgery Part B: Skull Base, Table of Contents

J Neurol Surg B Skull Base 2018; 79(04): 407-412
DOI: 10.1055/s-0037-1615750

Original Article

Georg Thieme Verlag KG Stuttgart · New York

Orbital Paraganglioma: A Systematic Review

Authors

Natalie Huang

¹Department of Otolaryngology – Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States
Hani M. Rayess

¹Department of Otolaryngology – Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States
Peter F. Svider

¹Department of Otolaryngology – Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States
Nadim M. Rayess

²Roski Eye Institute, Keck School of Medicine of USC, Los Angeles, California, United States
Adam Folbe

³Department of Otolaryngology – Head and Neck Surgery, William Beaumont Hospital, Royal Oak, Michigan, United States

⁴Barbara Ann Karmanos Cancer Institute, Detroit, Michigan, United States
Paul D. Langer

⁵Department of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey, United States
Jean-Anderson Eloy

⁵Department of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey, United States

⁶Department of Otolaryngology – Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, United States

⁷Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, United States

⁸Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Newark, New Jersey, United States
Michael Carron

¹Department of Otolaryngology – Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States

⁹Section of Otolaryngology, Department of Surgery, John D. Dingell VA Medical Center, Detroit, Michigan, United States

¹⁰Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology – Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan, United States

Abstract

Purpose The purpose of this study was to perform a systematic review of the literature on orbital paragangliomas, evaluating clinical presentation, diagnosis, management patterns, and prognosis.

Methods The systematic review was conducted based on the principles described in the Preferred Reporting Items for Systematic Reviews and Meta-Analysis. PubMed, Cochrane databases, Embase, and Web of Science were searched for articles related to orbital paragangliomas. Inclusion criteria included English language articles with original reports on human subjects. Data on clinical presentation, diagnosis, treatment, and prognosis were collected.

Results Twenty-seven articles met inclusion criteria (28 total patients). The mean patient age was 37.1 years (range, 3–75 years); 13 (46.4%) patients were male. The most common presenting symptoms were proptosis (89.2%), visual acuity changes (67.9%), and extraocular muscle restriction (64.2%). Lesions were most commonly intraconal (92.9%). Single modality therapy was employed in 19 patients (67.9%), including excision in 12 patients (42.9%) and exenteration in 5 patients (17.9%). Ten patients (35.7%) developed recurrence, and there was a 92.9% survival rate (mean follow-up, 29 months).

Conclusion Orbital paragangliomas are rare tumors with an excellent prognosis. These lesions commonly present with proptosis, and are primarily managed surgically with simple excision, although exenteration and adjuvant radiation may be necessary for invasive tumors. This series is the largest and most comprehensive systematic review of orbital paragangliomas conducted to date.

Keywords

orbital paraganglioma - systematic review - PRISMA

Full Text

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