Giant congenital melanocytic nevi in a patient with brain structural malformations and multiple lipomatosis.
We present a 9-year-old boy diagnosed from birth with giant congenital melanocytic nevi. He had central structural brain malformations of hemimegalencephaly of the right frontotemporal lobe and left occipitoparietal lobe, choroid plexus hypertrophy, and a Dandy-Walker variant. In addition, he developed multiple lipomatoses. These lesions were cutaneous except for two at the cerebellopontine angles, which were present from birth. This patient represents a rarely documented example of two histopathologies resulting in serious complications. The diagnostic issues and histopathologic process are discussed.
Wieselthaler
Nicky A
NA
Department of Radiology, Red Cross Children's Hospital, Cape Town, South Africa.
van Toorn
Ronald
R
Wilmshurst
Jo M
JM
D001921:Brain / Q000473:pathology*; D002648:Child; D006801:Humans; D008069:Lipomatosis, Multiple Symmetrical / Q000150:complications* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008548:Melanosis / Q000151:congenital* / Q000473:pathology; D009508:Nevus, Pigmented / Q000150:complications / Q000151:congenital* / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications / Q000151:congenital* / Q000473:pathology