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Diffuse cutaneous neurofibroma - neurofibromatosis type 1

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Claudication. Incidental findings on CTA.

Patient Data

Age: 55 years
Gender: Female

Diffuse cutaneous soft tissue nodules of various size of the abdominal and pelvic walls, as well as the lower limbs, with a plaque-like elevation of the skin, suggestive of neurofibromas.

Normal opacification with no significant atheromatous disease of the abdominal aorta, iliac and femoral arteries, as well as the popliteal and leg arteries.

A saccular splenic artery aneurysm measuring 13 x 11 mm is noted.

Case Discussion

CT features of multiple soft tissue nodules of cutaneous location most consistent with diffuse cutaneous neurofibroma in a patient known for neurofibromatosis type 1 (NF1).

Unlike plexiform neurofibromas, diffuse cutaneous neurofibromas are not strongly associated with neurofibromatosis type 1, with only 10% of affected individuals having the condition.

 

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