Desmoplastic Fibroma


- See:
        - Bone Tumor Menu
        - Fibroma:
        - Fibromatosis:

- Discussion:
    - also known as desmoid tumor;
    - is a rare tumor that that can involve bone or soft tissue;
    - is locally aggressive but does not metastasize;
    - soft tissue involvement:
          - tends to arise from the abdomen, shoulder, and axilla;
          - rarely occurs distally;
          - noted to be associated w/ pregnancy;
    - osseous involvement:
          - it occurs primarily in young adults;
          - typically develops as aggressive stage 3 lesion;
          - it behaves similar to its soft tissue counter part, (Fibromatosis);
          - location: tibia & fibula are most common sites;
          - radiographs:
               - centrally located metaphyseal or diaphyseal lesion;
               - lesion is surrounded by a thin margin of minimally reactive bone, and may have a trabeculated appearance;
               - tumor may go on to penetrate the thin cortical shell, but eventually it extends through the cortex into soft tissues;
          - differential diagnosis:
               - giant cell tumor of bone
               - fibrosarcoma of bone;

- Histology:
    - dense & irregularly arranged collagen bundles w/ infrequent fibrocytes;
    - may resemble fibromatosis;
    - mitoses, vascularity, and necrosis are unusual microscopic findings;
    - differential diagnosis: fibrosarcoma of bone;


- Treatment:

    - treatment of choice is excision with a wide margin;
    - recurrence after curettage is frequent, with invasion of soft tissue;
    - in the study by Pritchard et al 1996, there were recurrences in 2/13 patients that had wide resection vs 9/19 patients that had a marginal resection;
    - radiation therapy:
            - serves as a useful adjunct to surgical resection;
            - radiation may be indicated by itself for inoperative tumors, or residual disease after operative debulking



Desmoplastic fibroma of bone. A report of eight cases and review of the literature.

Musculoaponeurotic fibromatosis. A report of 28 cases and review of the literature.

Extra-abdominal desmoid tumors: a report of 234 cases.

Desmoid tumors in childhood

Local Control of Extra-Abdominal Desmoid Tumors  



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Friday, June 1, 2012 2:58 pm