Fibromatosis




Extraabdominal Desmoid Tumor, Aggressive Fibromatosis


CLINICAL SUMMARY


Fibromatosis is a benign, locally aggressive tumor originating in an intermuscular location, with a highly infiltrative growth pattern and a propensity to recur locally after surgical excision. Although it has no metastatic potential, it can cause significant morbidity. Surgery and radiation are used for treatment, while chemotherapy is used to treat multiple local recurrences.


DIAGNOSTIC FEATURES


















History


  • Young adults, with a median age in the 30s



  • Two to three times more common in females



  • Painful, enlarging mass



  • Loss of motion if near a joint

Location


  • The most common locations are shoulder girdle, back, chest/abdominal wall, and thigh



  • Tumors grow along the muscular aponeurosis



  • Recurrences can appear far from the original tumor

MRI


  • Distinctive bandlike regions of low signal on T1 and T2



  • Variably predominant high signal on T2



  • Indistinct margins, consistent with the invasive nature of the tumor




IMAGING

























  • CT scan demonstrates an abdominal wall fibromatosis, a location that is often associated with pregnancy ( left ). The lesion is slightly denser than the surrounding muscle, consistent with the prominent collagenization of the tumor.



  • CT scan shows a large, exophytic, proximal thigh fibromatosis ( right ).





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