Angiotropic intravascular large-cell lymphoma (malignant angioendotheliomatosis): Report of a case and review of the literature

We present a case of angiotropic large-cell lymphoma and review the literature in order to define the neurologic features of this rare disorder. This is the first report of gadolinium-DTPA imaging in angiotropic large-cell lymphoma that demonstrates infarcts of multiple ages, as well as striking meningeal enhancement. Angiotropic large-cell lymphoma should be suspected in patients with clinical evidence of small and large cerebral vessel disease and diagnosis requires skin, liver, renal, meningeal, or brain biopsy. Single modality treatment, using either radiation therapy or steroids, has been ineffective, and new findings of a lymphomatous origin of this neoplasm suggest that combination chemotherapy may be indicated.