Presentation
Right gluteal swelling
Patient Data
Age: 40 years old
Gender: Female
From the case:
Gluteal desmoid-type aggressive fibromatosis
{"current_user":null,"step_through_annotations":true,"access":{"can_edit":false,"can_download":true,"can_toggle_annotations":true,"can_feature":false,"can_examine_pipeline_reports":false,"can_pin":false},"extraPropsURL":"/studies/110693/annotated_viewer_json?lang=us"}
Right gluteal deep fairly defined soft tissue mass lesion is seen deep to the right gluteal muscles. It's smoothly abutting the adjacent proximal femoral shaft. The mass elicits heterogeneous signal with internal bands of persistent low signal on all pulse sequences. The lesion shows moderate enhancing areas in the non-dark cellular areas.
Case Discussion
This is a pathologically proven case of desmoid-type fibromatosis.
Our case presented typical MRI features of desmoid fibromatosis including:
- typically a reduced signal on T2 WI secondary to dense collagen and hypocellularity
- low signal intensity non enhancing bands corresponding to high collagenous stroma (known as the band sign) are seen in all pulse sequences, however, this sign is not specific as it could be seen in the giant cell tumor of the tendon sheath or myxofibrosarcoma
- moderate to marked enhancement mainly in the non-fibrotic high cellular components of the mass
- a rim of surrounding fat giving the split fat sign
Desmoid-type fibromatosis or aggressive fibromatosis is an aggressive fibroblastic tumor.
Desmoid type fibromatosis is classified according to its location into three types:
- intra-abdominal desmoid
- abdominal wall desmoid
- extra-abdominal desmoid (commonest locations include extremities, head and neck regions, chest wall, breast, and gluteal region)
Unable to process the form. Check for errors and try again.