Intraorbital optic nerve hemangioblastoma with von Hippel-Lindau disease

Abstract

An intraorbital hemangioblastoma of the optic nerve is reported in a 23-year-old woman with von Hippel-Lindau disease. The absence of dural attachment and the microscopic findings were characteristic of hemangioblastoma. Four years later the patient developed a cystic hemangioblastoma in the left cerebellum which was successfully treated.

Hemangioblastoma of the optic nerve

“…Progressive growth of intraorbital tumors causes axial proptosis, also a moderately common presenting feature present in 26%, with one patient noted to have pulsatile proptosis. 16 Proptosis is generally reversible with surgery; however, when optic nerve function is compromised, it is usually permanent. The most common sign on initial examination was optic atrophy, seen in 37% of patients.…”

Hemangioblastoma of the Optic Nerve-Case Report-

“…Optic nerve hemangioblastoma is mainly supplied by the ophthalmic artery and drained by dilated veins around the tumor. 2,4,7,9,11,13,17) These findings suggest that hemangioblastoma arises from the optic nerve tissue rather than the optic nerve sheath, 3,5,12) and may be useful to differentiate hemangioblastoma from meningioma and schwannoma. Histological examination shows that optic nerve hemangioblastoma usually does not have a capsule, 9,16,17) in contrast to meningioma.…”

“…1) Only 15 cases of optic nerve hemangioblastoma including the present case have been reported (Table 1). [2][3][4][5][7][8][9][11][12][13][14][15][16][17] Two controversial cases were excluded because the diagnosis was unclear. 8,13) The seven male and eight female patients were aged from 15 to 64 years (median 30 years, mean 33.3 years).…”

Angiomatosis retinae

“…In et al[56] stellten bei einer 23-jährigen Frau mit einem VHL ein intraorbitales Hämangioblastom fest. Das Sehvermögen links verschlechterte sich, es trat eine Protrusio bulbi auf.…”