Publication:

Asymptomatic Sturge-Weber syndrome patient with extradural-intradural Ewing sarcoma

Sturge Weber syndrome (SWS), also called as encephalotrigeminal angiomatosis is an uncommon congenital neurological disorder and frequent among the neurocutaneous syndromes specifically with vascular predominance. Symptoms and signs depend on the extent and location of the venous dysplasia. In its complete trisymptomatic form, SWS is physically characterized by port-wine stains (PWS) over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. We report a case of 19 year old gentleman asymptomatic Roach type II SWS patient with Ewing sarcoma presenting as extradural-intradural spinal tumour.