Total resection of giant plexiform neurofibroma of the entire back in one stage by autologous tumor skin graft

摘要

The patient was a thirteen-year-old girl who had many smooth brown freckling spots of various sizes (the diameter of the seven spots exceeded 15 mm) scattered on her body. There was a lesion on her back that had slowly grown over time and quickly in the past three years. The histological examination in a local hospital confirmed the diagnosis of neurofibromatosis type I without malignant transformation. The patient had no history of seizures or learning disabilities. The size of the large neurofibroma on the back extended into the midaxillary line and measured 42 x 48 x 5 cm3 [fig1 left]. An MRI showed a diffuse neurofibroma with associated dysplastic blood vessels exhibiting irregular areas of tunica media and sinusoidal-like vascular channels (pseudo-hemangioma) [Figure 1 middle]. An angiography demonstrated that the blood vessels of the tumor originated from the intercostal arteries and the transverse cervical artery.