%0 Journal Article %T An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex: A case report %A Santosh Rai %A Piyush Kalakoti %A MM Syed %A Purujit J Thacker %A Rishi Jain %A Gaurav Kalra %J Journal of Medical Case Reports %D 2011 %I BioMed Central %R 10.1186/1752-1947-5-267 %X In this report, we describe the case of a 16-year-old Indian girl who presented to our hospital with swelling on the scalp which had progressed from the hairline to just above the left brow, causing mechanical ptosis. She was born with a black-pigmented triangular patch covered with hair over the scalp which had increased in size over a period of eight years after birth. An X-ray of her skull and ultrasonography revealed soft tissue swelling in the left temporofrontoparietal region. Magnetic resonance imaging of her brain showed the presence of 8.99 cm กม 2.26 cm abnormal signal intensity involving the scalp, a few small tubers with cortical dysplasia in the left frontoparietal region with asymmetric dilatation, and the presence of calcified subependymal nodules within the left lateral ventricle. These findings were suggestive of tuberous sclerosis. A histopathological examination of the swelling was suggestive of congenital melanocytic nevi. The patient underwent surgery. Excision of the tumor with primary skin grafting was done, with the graft being taken from the medial aspect of the right thigh.This case warrants further research to provide concrete evidence of an association of neurocutaneous melanoma with tuberous sclerosis complex. Research should be conducted to prove whether this is an unusual association or a new syndrome. Also, similar cases in other parts of the globe should be documented, as they would provide substantial support for such an association.Congenital melanocytic nevi (CMN) are found in approximately 1% of newborn infants, but 90% of these nevi are very small. Giant congenital nevi (GCN), giant hairy nevi and nevocellular nevi represent a special group of melanocytic lesions that generally cover large areas of the body and carry a potential risk for the development of malignant melanoma [1]. A congenital nevus is one of several known risk factors leading to the development of melanoma. Fortunately, melanoma remains an uncommon malignancy in %U http://www.jmedicalcasereports.com/content/5/1/267