Neoplastic angioendotheliosis: a case with spontaneous regression and radiographic appearance of cerebral arteritis.
Neoplastic angioendotheliosis (NA) of the CNS is usually characterized by systemic vascular disease and a rapidly fatal course. We report a 52-year-old woman with dementia, spasticity, and sensory deficits developing insidiously over a year. Diagnostic findings included small CT lucencies in the brain and angiographic irregularities of medium-sized arteries (resembling cerebral arteritis). Brain biopsy revealed numerous small infarcts as well as pleomorphic, highly malignant tumor cells within cerebral meningeal vessels. Without treatment, she experienced only slight increase of dementia before death from pneumonia. At autopsy, there was almost complete regression of the intravascular cerebral tumor. The clinical course was unusual for the length of illness and the radiographic picture of cerebral vasculitis. Clinical features often present with NA--such as strokelike events, elevated sedimentation rate, renal impairment, and fever--were notably absent.
D001927:Brain Diseases / Q000175:diagnosis; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D014657:Vasculitis / Q000175:diagnosis