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A 37-year-old woman presented with left eye pain, headaches, and vision loss. MRI showed a left sphenoid mass, with optic nerve compression and proptosis (figure 1). The left eye had minimal reactivity to light, scleral erythema, and proptosis. A metastatic lesion or a lymphoma was suspected; however, pathology showed an epithelioid hemangioendothelioma (figure 2). There was no extracranial disease. Intracranial epithelioid hemangioendothelioma is rare, with around 40 reports. Thirty-two percent show local invasion, mortality is 15%, 24% recur, and 15% metastasize.1 It is associated with the WWTR1/CAMTA1 fusion protein.2 The treatment is surgery, with unclear roles for adjuvant therapy.1

Intracranial epithelioid hemangioendothelioma causing subacute loss of vision