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 × 48 × 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. The maximum diameter of these feeding and draining vessels was 1 cm, and a distinct plane with main vessel perforators [Figure 1 middle] and nerve bundles between tumor stroma and deep musculofascial plane could be identified [Figure 1 right]. Interventional radiologists were unable to embolize such complex vasculature. Three days before the operation, 400 mL of autologous blood was obtained for perioperative transfusion.
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Published online: August 22, 2011
© 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Inc. All rights reserved.