Periosteal desmoid tumor


Periosteal desmoid tumor

Description, Causes and Risk Factors:

Periosteal desmoid is a tumor-like fibrous proliferation of the periosteum at the musculotendinous attachment of the adductor magnus or the medial head of the gastrocnemius muscle on the posteromedial cortex of the distal femur. Because of its location, it also goes by the following names: distal metaphyseal femoral defect or medial supracondylar defect of the femur.

The etiology is thought to be related to repetitive trauma, similar to tug lesions elsewhere in the body. It is, therefore, also called an avulsive cortical irregularity. Although the term periosteal desmoid is widely accepted, it has an unfortunate similarity to the term desmoid tumor of soft tissue.

Periosteal desmoid usually occurs between the ages of 12 and 20 years, mainly in boys. There is no significant racial or ethnic distribution.

In the United States, approximately 900 people are diagnosed with periosteal desmoid tumors every year.

Symptoms:

Symptoms include:

    Local pain and tenderness around the knee.

  • Difficulty in walking and doing regular activities.

Diagnosis:

Blood test includes hemoglobin (Hb/Hgb) level, white blood cells (WBCs), erythrocyte sedimentation rate (ESR), and serum biochemistry.

Radiographic findings include a saucer-like cortical defect at the posteromedial cortex of the distal femur. The base is usually sclerotic, but may be poorly defined and irregular. Small bony spicules at the cortical surface may simulate an aggressive lesion.

Scintigraphy is usually normal, but focal increase in activity can occasionally be seen.

MRI reveals a T1-hypointense, T2-hyperintense lesion with a hypointense base at or near the attachment of the adductor magnus or medial head of the gastrocnemius muscles. Periosseous and marrow edema may also be seen.

Histopathologic examination of the lesion demonstrate fibroblastic spindle cells that produces large amount of collagen.

Treatment:

If you are diagnosed with a desmoid tumor it is recommended that you be evaluated in a major academic hospital. Patients with periosteal desmoid tumors should be evaluated by an orthopedist and oncologist. In more severe cases Multidisciplinary approach is indicated. Surgery followed by radiation and medical therapy is the most commonly used treatment is majority of cases.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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