Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review

A 63-year-old woman developed gradual slowness, recurrent multifocal deficits, severe constitutional symptoms and hypopituitarism which progressed to death over 2.5 months. Elevation in lactico dehydrogenases was the main biological abnormality. Necropsy showed an intravascular malignant proliferation which proved to be a lymphoma of B-lineage. In order to better define diagnostic criteria, we reviewed previously reported cases of intravascular lymphomatosis of the central nervous system. A strategy for establishing the diagnosis is proposed.