Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome

Abstract

A man in his 20s presented to the neurosurgery department 2 years ago with headache and blurred vision. He was diagnosed to have a suprasellar mass on neuroimaging. Best-corrected visual acuity in the right eye was 6/36 and that in the left eye was 6/60. Automated visual fields showed a temporal hemianopia in the right eye and an advanced field defect in the left eye. His hormonal profile was normal, and he underwent partial excision of suprasellar tumour, which was a histopathologically proven paraganglioma (PGL). Subsequently, the patient underwent radiotherapy and his vision and visual fields showed improvement. Follow-up examination 3 years later showed a left retinal capillary hemangioblastoma (RCH), which was treated with green laser photocoagulation, resulting in complete sclerosis. This case is unique because of the extremely rare coexistence of a sellar PGL and RCH, which to our knowledge has not been reported so far.

Case Report: Paraganglioma in the sellar region: longitudinal observation and surgical outcome

“…Although paragangliomas are generally considered to be indolent entities corresponding histologically to World Health Organization (WHO) grade I ( 5 ), a high recurrence rate following surgical resection (10% for paragangliomas of carotid body and 50% to 60% for those occurring in other sites) and even metastasis (~10%) have been reported ( 6 ). Previously, we reported a consecutive surgical series of 19 patients with pathologically diagnosed spinal paragangliomas, in which we noted a considerable risk of in-situ recurrence Sellar paragangliomas are extremely unusual as paraganglia do not normally exist in this region, which has been only reported in very limited cases ( 7 – 36 ). This may be secondary to the persistence of paraganglionic tissue caused by deficient involution during early life.…”