Optic Nerve Haemangioblastoma: Signs of Chronicity

Abstract

Optic nerve haemangioblastomas remain exceedingly rare extrinsic tumours of the optic nerve, often associated with von Hippel-Lindau disease. The authors report a 25-year-old female with a slowly progressive unilateral optic nerve lesion, causing reduced vision and bilateral optic tract oedema. A diagnosis of optic nerve haemangioblastoma with piloid gliosis was made histologically after surgical resection. This is the first reported case of such dual pathology occurring in the optic nerve. The patient has been monitored without further adjuvant treatment, and has not had a recurrence to date, at 6 years of follow-up.

Hemangioblastoma of the optic nerve

“…5,14 A more contemporary finding associated with cerebral and optic nerve hemangioblastomas is peritumoral edema. 3,24,38 As retrobulbar hemangioblastomas are often supplied by branches of the ophthalmic artery, the high blood volume and permeability of these tumors results in a disproportionately large amount of edema for the size of the lesion. 25 This edema tends to propagate along low-resistance white matter pathways, and can be demonstrated on serial imaging.…”

“…7 A recent report by McGrath and coauthors demonstrated Rosenthal fibers with surrounding piloid gliosis in an ONH as a marker for chronicity. 24 Gliosis can accompany neural lesions, with a subset of astrocytic gliosis characterized by elongated, granular, hypereosinophilic, deposits called Rosenthal fibers (Figure 3c). 24 This appearance can be termed 'piloid gliosis', given that it mimics features seen in pilocytic astrocytoma.…”

“…24 Gliosis can accompany neural lesions, with a subset of astrocytic gliosis characterized by elongated, granular, hypereosinophilic, deposits called Rosenthal fibers (Figure 3c). 24 This appearance can be termed 'piloid gliosis', given that it mimics features seen in pilocytic astrocytoma.…”

“…In 1988 Nerad and coworkers were able to excise an intraorbital ONH via a lateral orbitotomy approach, a technique which was also employed by McGrath and colleagues in 2018. 24,27 Other contemporary studies advocate an endoscopic transsphenoidal approach or craniotomy. The endoscopic approach has shown mixed outcomes, with some tumors not able to be fully excised and evidence of postoperative visual decline.…”

CT and MRI Characteristic Findings of Sporadic Renal Hemangioblastoma: Two Case Reports and Review of the Literature

“…Beyond the CNS, hemangioblastomas can also occur in the peripheral nervous system [7,8], retroperitoneum [9,10], pelvic cavity [11], soft tissue [12], bone [13], adrenal glands [14], lung, and liver [15].…”