척추측만증과 거대 밀크커피색 반이 동반된 혈관성 총상 신경섬유종

초록

Plexiform neurofibromas are benign proliferations arising from the peripheral nerves and represent one of the diagnostic features of neurofibromatosis type 1. We present a case of a 14-year-old boy, who had large vascular neurofibromatous elephantiasis associated with a giant café-au-lait patch on his lateral aspect of the left hemithorax. Although he had no perceptible symptoms, radiologic examination revealed mild thoracic scoliosis with left side convexity. Our treatment of the skin lesion was one-stage palliative tumor debulking.

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