Neurofibromatosis type 1 with pedunculated neurofibromas

Case contributed by Ciro Menna Barreto Duarte
Diagnosis almost certain

Presentation

Long-standing condition.

Patient Data

Age: 70 years
Gender: Female

CT scout and surface shaded images demonstrate innumerable cutaneous neurofibromas.

There is a right paravertebral lesion (neurofibroma), seen on the axial CT images.

Scoliosis is also noted. 

Case Discussion

NF1, also known as von Recklinghausen disease or peripheral neurofibromatosis, is characterized by a progressive disease that affects the growth of neural tissues and has varied manifestations, such as numerous tumors of peripheral nerves, skin manifestations, ocular disorders, gliomas, and dysplastic skeletal lesions.

Skin tumors can take on sessile or pedunculated forms. The second form can reach great dimensions, as in this case.

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