SKULL BASE TUMOR IN A PATIENT WITH PHACOMATOSIS PIGMENTOVASCULARIS

• Published in: Brain pathology (Zurich, Switzerland) Vol. 21; no. 6; pp. 705 - 708
• Main Authors: Carvalho, Carlos H., Batista, Leonardo M, Bornemann, Antje, Acioly, Marcus André, Tatagiba, Marcos
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.11.2011
• Subjects: Correspondence; Humans; Male; Meningeal Neoplasms - complications; Meningeal Neoplasms - pathology; Meningeal Neoplasms - surgery; Middle Aged; Neoplasms, Multiple Primary - pathology; Neoplasms, Multiple Primary - surgery; Neurilemmoma - complications; Neurilemmoma - pathology; Neurilemmoma - surgery; Neurocutaneous Syndromes - complications; Neurocutaneous Syndromes - pathology; Neurosurgical Procedures; Nevus, Blue - complications; Nevus, Blue - pathology; Nevus, Blue - surgery; Skull Base Neoplasms - complications; Skull Base Neoplasms - pathology; Skull Base Neoplasms - surgery
• ISSN: 1015-6305; 1750-3639
• DOI: 10.1111/j.1750-3639.2011.00528.x

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.