Báo cáo y học: " An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex: A case report"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex: A case report. | Rai et al. Journal of Medical Case Reports 2011 5 267 http content 5 1 267 JOURNALOF medical Ur Case REPORTS CASE REPORT Open Access An unusual case of congenital melanocytic nevus presenting as neurocutaneous melanoma coexisting with Tuberous Sclerosis complex A case report 1 2 2 2 1 1 Santosh Rai Piyush Kalakoti MM Aarif Syed Purujit J Thacker Rishi Jain and Gaurav Kalra Abstract Introduction Congenital melanocytic nevi are among the several known risk factors for the development of melanoma. Neurocutaneous melanosis is a rare congenital non-hereditary disorder characterized by the presence of multiple and or giant congenital melanocytic nevi. It is a rare condition with fewer than 200 cases reported in the literature. Its association with tuberous sclerosis complex a form of the neurocutaneous syndrome is an unusual finding which to the best of our knowledge has not been documented in the English literature so far. Herein we present the first case documenting such an association in a 16-year-old post-pubertal Indian girl. Case presentation 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 cm X 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 .

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