IMPORTANT: We currently have a number of bugs related to image cropping and are actively trying to resolve them. In the meantime, we have disabled cropping. Apologies for any inconvenience. Stay informed: radiopaedia.org/chat

Plexiform neurofibroma of perineum and pelvis

Case contributed by Kareem Mohamed
Diagnosis probable

Presentation

Perenial swelling for months. No constitutional symptoms.

Patient Data

Age: 10 years
Gender: Male

Initial ultrasound scan showed multiple skin and subcutaneous hypoechoic soft tissue nodules scattered at the right gluteal and perineal regions with faint vascularity noted within.

Pelvis MRI with contrast

mri

An ill-defined infiltrative soft tissue lesion is seen centered at the right perineal region extending to right gluteal muscles, medially limited by midline and associated with right sided sacral intraspinal extension mainly along right S2 nerve root.
The lesions elicit low signal in T1, heterogeneous high signal in T2 and STIR with faint heterogeneous post contrast enhancement.

Case Discussion

The cutaneous and subcutaneous location of the skin lesions and deep soft tissue extension with limitation of the extension by the midline and associated bulky sacral nerve root gives the impression of dermatomal distribution in this case.

The main differential in this age group is a venous malformation, yet lack of intense post-contrast enhancement, moderately hyperintense signal in T2 and STIR as compared to that seen in hemangiomas as well as the lack of dominant tortuous supplying vessels rendered this possibility to be less likely.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.