Aggressive fibromatosis

Case contributed by Enzo Gabriel Redolfi Mema
Diagnosis certain

Presentation

On regular follow-up and treatment monitoring for aggressive fibromatosis, diagnosed during surgery almost 10 years ago; he presented with an increase in diameter and pain in the left thigh at the time.

Patient Data

Age: 45 years
Gender: Male
mri

There is a large low signal intensity mass lesion extending from the pelvis to the thigh, knee and proximal leg, with heterogeneous enhancement in the posterior soft parts of the left upper leg.

The lesion slightly displaces the adjacent muscles especially the hamstring muscles and the medial gastrocnemius muscle in the lower leg it shows close contact with the vascular structures and nerves with displacement of the same in some sectors. It also displays close contact with the bony structures including the femoral diaphysis and condyles, the posterior tibial plateau and the proximal fibula.

Fatty changes are observed in the left soleus, flexor hallux longus, and tibialis posterior muscles; which suggests chronicity of the injury. 

Cystic lesion in the proximal tibiofibular joint probably in keeping with a ganglion cyst.

In the present case, after the first surgical approach, marked growth or progression of the disease was observed, given the usual behaviour of the aforementioned entity.

Therefore, it was decided to treat the patient conservatively with immunotherapy and follow-up, observing the stability of the disease.

This is the patient's 8th control MRI, without changes compared to the previous postsurgical examinations.
In the case of our patient, follow-up examinations were performed with T1, T2, and STIR sequence and to evaluate activity, T1 fat sat with contrast was chosen for visualising vascularisation and its behaviour, meaning that in phases of greater activity, "we expected to find greater enhancement ".

Case Discussion

This is a case of aggressive fibromatosis with marked growth after the first surgical approach and typical imaging features. The initial treatment was surgical resection without clear margins since the aetiology of the disease was not known at the time, which conditioned the progression of the disease and its more aggressive behaviour.

Because of the above, we suggest being particularly cautious in the presence of soft tissue injuries, as it cannot be ruled out that they are due to desmoid injury. In this case, we recommended starting the workup of the soft tissue mass with ultrasound-guided sampling.

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.