The gross and microscopic features of an unusual case of neurocutaneous melanosis are described. This is an example of the rare association between a primary central nervous system malignant melanoma and a giant congenital melanocytic naevus of 'bathing trunk' distribution.

Sturge-Weber Syndrome (SWS) occurs sporadically with a frequency of approximately 1 in 50,000. SWS is a mesodermal phakomatosis. Klippel-Trenaunay Weber syndrome (KTWS) is another very rare phakomatosis. Overlap between SWS & KTWS is very rarely encountered. We report a 19 months old boy with features of both SWS and KTWS. The reported case had seizures, port wine haemangioma of the right side ...

Neurocutaneous melanosis (NCM) is a rare neuroectodermal dysplasia characterized by large or multiple cutaneous congenital pigmented nevi and benign or malignant melanocytic tumors of the leptomeninges. Although the MR manifestations of this disease have been reported in a small series of cases, the usefulness of fluid-attenuated inversion recovery (FLAIR) MR findings has not been documented. W...

Epidermal Nevus Associated with Cerebral and Ocular MR Imaging Abnormalities This letter focuses on Schimmelpenning syndrome (SS), a neurocutaneous disorder related to epidermal nevus syndromes and characterized by craniofacial nevus, neurologic anomalies, and ocular pathology. We discuss clinical and MR imaging features of a 10-year-old boy, pointing out the etiopathologic substratum of this c...

Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is the most frequent neurocutaneous syndrome, characterised by vascular malformations which manifest sporadically. The full presentation includes brain vascular malformations (pial or leptomeningeal angioma), cutaneous abnormalities (facial angioma) and ocular abnormalities (choroidal angioma). We present the case of a patient wi...