Neoplastic angioendotheliomatosis
H. G. Raroque Jr, R. N. Mandler, M. S. Griffey, W. W. Orrison and M. Kornfeld
Department of Neurology, University of New Mexico School of Medicine, Albuquerque 87131.
A 60-year-old white man presented with aphasia, seizures, paraparesis, and
incontinence. His serologic and hematologic profiles were unremarkable. His
cerebrospinal fluid showed pleocytosis, increased daily central nervous
system IgG synthesis, increased myelin basic protein, and negative cytology
and cultures. Cerebral computed tomography exhibited multiple areas of
hypodensity but spinal computed tomography and myelography showed no
abnormalities. Cranial and spinal magnetic resonance imaging revealed areas
of increased signal on T2-weighted images. The use of gadolinium-pentetic
acid on T1-weighted images delineated smaller areas of cortical enhancement
with surrounding rim of decreased signal. Brain biopsy showed intravascular
malignant cells positive for leukocyte common antigen and B-cell markers.
The diagnosis was neoplastic angioendotheliomatosis (intravascular
lymphomatosis). To our knowledge, this is the first report on the use of
both cranial and spinal magnetic resonance imaging in this condition.
