Desmoid



Desmoid


Michael P. Federle, MD, FACR

Kathleen E. Jacobs, BA









(Left) Axial CECT in a 35-year-old man with Gardner syndrome who had a colectomy at age 30 shows the suture line image for the ileoanal pouch that was anastomosed to the anus. (Right) Axial CECT in the same patient shows a small soft tissue density mesenteric mass image, a typical desmoid in this setting. This caused no symptoms and was not resected.






(Left) Axial CECT in the same patient 8 months later shows rapid growth of the mesenteric desmoids image. Note the encasement of the mesenteric vessels image. (Right) Axial CECT in the same patient shows the mesenteric desmoid encasing mesenteric vessels image and the bowel. The patient was treated with complete resection of the small bowel and mesentery followed by small bowel transplantation.



TERMINOLOGY


Synonyms



  • Aggressive fibromatosis


Definitions



  • Rare, benign, locally aggressive, nonencapsulated tumor of connective or fibrous tissue


IMAGING


General Features



  • Best diagnostic clue



    • Small bowel mesentery or abdominal wall mass arising from scar of prior surgery


  • Location



    • Abdominal



      • Mesentery: Small bowel (most common)


      • Musculature: Rectus, internal/external oblique, psoas, pelvic (rare)


      • Retroperitoneum


    • Extraabdominal



      • Bladder, ribs, and pelvic bones


  • Size



    • Mass may range from 4-20 cm


  • Morphology



    • Well- or ill-defined, tan or white, hard fibrous mass



      • Clear, lobulated margin (75%); ill-defined, infiltrative (25%)


  • Key concepts



    • Abdominal desmoids can be solitary or multiple


    • Locally aggressive mesenteric primary tumor


    • Tend to arise in musculoaponeurotic planes


    • Tend to invade locally and recur and grow very rapidly, especially in Gardner syndrome


    • May involve bowel loops, bladder, ribs, pelvic bones


    • Sometimes classified as low-grade fibrosarcoma or subgroup of fibromatosis


    • Usually associated with Gardner syndrome



      • Familial polyposis coli, osteomas, dental defects, congenital pigmented lesions of retina


      • Epidermoid (sebaceous) cyst and fibromas of skin


      • Periampullary, adrenal, thyroid, and liver carcinomas


    • 75% of patients with desmoid tumors have had prior abdominal surgery


    • 18-20% of patients with Gardner syndrome develop desmoids



      • Accounts for 45% of fibrous lesions in Gardner

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Jun 8, 2016 | Posted by in GENERAL RADIOLOGY | Comments Off on Desmoid

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