Skull base tumor in a patient with phacomatosis pigmentovascularis.

A 58-year-old man with clinical diagnosis of phacomatosis pigmentovascularis (PPV) experienced tinnitus and progressive hearing loss due to a jugular foramen tumor.Attached to the tumor capsule, were several pigmented spots. Pathological examination revealed a tumor composed by two different tissues, namely a Schwannoma grade I associated with a leptomeningeal blue nevus. The neuropathological aspects of this unusual association are discussed. The association of PPV with a pigmented skull base tumor has not been described to date and illustrates the importance of systemic examination in PPV.