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

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 hem...

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Bibliographic Details
Published in:BMJ case reports 2022-03, Vol.15 (3), p.e245907
Main Authors: Ghaisas, Shamika, Rao, Kavitha Shekar, Preethi, Archana, Rani, Padmaja Kumari
Format: Article
Language:English
Subjects:
Abdomen
Case reports
Cysts
Family medical history
Hemangioblastoma - diagnosis
Humans
Lasers
Male
Medical imaging
Mutation
Neuroendocrine tumors
Neurosurgery
Paraganglioma - complications
Paraganglioma - diagnosis
Paraganglioma - surgery
Retina - pathology
Retinal Neoplasms - diagnosis
Tumors
Veins & arteries
von Hippel-Lindau Disease - complications
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Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2021-245907