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9th North Ward, 33 Badachu Road, Plastic Surgical Hospital, Affiliated with Peking Union Medical College, Shijingshan District, Beijing 100041, People’s Republic of China
9th North Ward, 33 Badachu Road, Plastic Surgical Hospital, Affiliated with Peking Union Medical College, Shijingshan District, Beijing 100041, People’s Republic of China
9th North Ward, 33 Badachu Road, Plastic Surgical Hospital, Affiliated with Peking Union Medical College, Shijingshan District, Beijing 100041, People’s Republic of China
9th North Ward, 33 Badachu Road, Plastic Surgical Hospital, Affiliated with Peking Union Medical College, Shijingshan District, Beijing 100041, People’s Republic of China
9th North Ward, 33 Badachu Road, Plastic Surgical Hospital, Affiliated with Peking Union Medical College, Shijingshan District, Beijing 100041, People’s Republic of China
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.
Figure 1left: The giant back neurofibromatosis preoperation (up); middle: Dysplastic blood vessels exhibiting irregular areas of matrix and sinusoidal-like
vascular channels and main vessel perforators feeding tumor tissue from intercostal
artery; right: Distinct plane can be distinguished between tumor stroma and deep musculofascial
plane.
