[Neoplastic angioendotheliosis presenting Tolosa-Hunt syndrome, intraspinal invasion and intraorbital tumor].

A 76-year-old woman with painful erythema on legs and right oculomotor disturbance was diagnosed as neoplastic angioendotheliosis (NAE, B cell type) by skin biopsy. Remission was obtained by 2 courses of VEPA regimen. But 3 months later, she relapsed with right oculomotor, optic, trochlear and trigeminal nerve disturbance which suggested that she had Tolosa-Hunt syndrome. Cerebrospinal fluid (CSF) revealed infiltration of lymphoma cells. She developed intraorbital tumor and died of respiratory failure due to tumor emboli. Autopsy revealed infiltration of tumor cells in the small vessels of systemic organs. This rare case presented intracerebrospinal and intraorbital tumor growth in addition to intravascular infiltration of tumor cells.