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. 2015 Sep;2(1):36-9.
doi: 10.1159/000436973. Epub 2015 Jul 15.

Orbital Paraganglioma and Succinate Dehydrogenase Staining for Genetic Testing Triage and Prognosis

Affiliations

Orbital Paraganglioma and Succinate Dehydrogenase Staining for Genetic Testing Triage and Prognosis

Cesar M Salinas-La Rosa. Ocul Oncol Pathol. 2015 Sep.

Abstract

Purpose of the study: To describe the rare occurrence of a paraganglioma in the orbit and how to triage for genetic testing and assess the prognosis with succinate dehydrogenase subunit B (SDHB) immunohistochemical staining.

Method: Case report.

Procedures: A 47-year-old 'healthy' male presented with painless exophthalmos and diplopia secondary to an infraorbital tumour mass.

Results: The orbital biopsy was diagnosed as paraganglioma with positive staining with SDHB.

Conclusion: The rarity of an orbital paraganglioma was followed by the clinical search for a possible occult extraorbital primary paraganglioma. SDHB staining helped in the triage for genetic testing and gave an idea about the prognosis for this tumour.

Keywords: Familial syndrome; Orbit; Paraganglioma; Succinate dehydrogenase immunohistochemical stain.

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Figures

Fig. 1.
Fig. 1.
a-g Orbital paraganglioma. a CT scan with an enhancing mass inferior to the right globe measuring 13 × 13 mm, abutting the right canal and causing mild upward globe displacement. b Nests of polygonal cells on a vascularised stroma, consistent with paraganglioma. HE. ×200. c Nests of polygonal cells on a vascularised stroma, consistent with paraganglioma. HE. ×400. d Paraganglioma cells positive for chromogranin immunohistochemical stain. ×400. e Paraganglioma cells positive for synaptophysin immunohistochemical stain. ×400. f Sustentacular cells highlighted with S100 immunohistochemical stain. g SDH immunohistochemical stain (red) with granular cytoplasmic staining in paraganglioma tumour cells with a positive internal control in endothelial cells (CD31, brown), a pattern of staining consistent with a positive result. ×400.

References

    1. Sharma MC, Epari S, Gaikwad S, Verma A, Sarkar C. Orbital paraganglioma: report of a rare case. Can J Ophthalmol. 2005;40:640–644. - PubMed
    1. Hill RH, 3rd, Platt SM, Bersani A, Barker-Griffith A, Strumpf K. Regression of a paraganglioma tumor of the orbit. Orbit. 2015;34:99–102. - PubMed
    1. Bedner MM, Trainer TD, Aitken PA, Grenko R, Dorwart R, Duckworth J, Gross CE, Pendlebury WW. Orbital paraganglioma: case report and review of the literature. Br J Ophthalmol. 1992;76:183–185. - PMC - PubMed
    1. Bagheri A, Aletha M, Salour H, Abdollahi A, Silbert D, Rezaei-Kanavi M. Orbital paraganglioma presenting as lateral rectus enlargement and its novel management: a case report and review of the literature. Orbit. 2012;31:256–260. - PubMed
    1. Makhdoomi R, Nayil K, Santosh V, Kumar S. Orbital paraganglioma: a case report and review of the literature. Clin Neuropatol. 2010;29:100–104. - PubMed