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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8094061 | PMC |
| http://dx.doi.org/10.1111/j.1750-3639.2011.00528.x | DOI Listing |
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