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DOI: 10.1148/rg.234025154
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Right arrow Musculoskeletal Radiology

Hemophilic Pseudotumor: Radiologic-Pathologic Correlation1

James M. Stafford, MD, Tina T. James, MD, Anton M. Allen, MD and Lisa R. Dixon, MD

1 From the Departments of Radiology (T.T.J., J.M.S., A.M.A.) and Pathology (L.R.D.), University of Tennessee Medical Center, Knoxville, 1924 Alcoa Hwy, Knoxville, TN 37920. Received October 16, 2002; revision requested November 25 and received January 10, 2003; accepted January 13. Address correspondence to J.M.S. (e-mail: jstaffor@mc.utmck.edu).



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Figure 1.  Photograph shows gross distortion of the lower left leg by multiple large masses. Bluish discoloration of the skin is also evident.

 


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Figure 2.  Axial T1-weighted MR image of the midthigh shows a large, heterogeneous soft-tissue mass occupying most of the central and anterior thigh. Only a small shell of the femur remains. Note the severe displacement and compression of the gracilis (thin arrow) and flexor compartment (arrowheads) muscles. The superficial femoral neurovascular bundle is displaced medially (thick arrow).

 


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Figure 3a.  Coronal T1-weighted (a) and T2-weighted (b) MR images of the thigh show a heterogeneous mass with a low-signal-intensity capsule. In general, the interior of the mass demonstrates high signal intensity on the T1-weighted image and low signal intensity on the T2-weighted image (white arrow), findings that are compatible with intracellular methemoglobin. Smaller areas of high signal intensity compatible with extracellular methemoglobin are seen on both images (arrowhead). Note also the areas of low signal intensity in a and high signal intensity in b representing serous fluid (black arrow).

 


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Figure 3b.  Coronal T1-weighted (a) and T2-weighted (b) MR images of the thigh show a heterogeneous mass with a low-signal-intensity capsule. In general, the interior of the mass demonstrates high signal intensity on the T1-weighted image and low signal intensity on the T2-weighted image (white arrow), findings that are compatible with intracellular methemoglobin. Smaller areas of high signal intensity compatible with extracellular methemoglobin are seen on both images (arrowhead). Note also the areas of low signal intensity in a and high signal intensity in b representing serous fluid (black arrow).

 


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Figure 4.  Axial fat-saturated fast spin-echo MR image of the knee region shows multiple heterogeneous soft-tissue masses that have completely destroyed all the bones about the knee and knee joint. The adjacent soft-tissue structures have been replaced or are severely compressed. Note also the small gastrocnemius muscles posteriorly (arrows).

 


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Figure 5a.  Anteroposterior radiographs of the thigh (a) and tibia-fibula (b) show a large soft-tissue mass with lytic destruction of the distal femur and proximal fibula. Severe disuse osteopenia is also present.

 


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Figure 5b.  Anteroposterior radiographs of the thigh (a) and tibia-fibula (b) show a large soft-tissue mass with lytic destruction of the distal femur and proximal fibula. Severe disuse osteopenia is also present.

 


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Figure 6.  Arterial phase digital subtraction angiogram of the proximal thigh shows splaying of the vessels around the upper aspect of the dominant pseudotumor. The soft-tissue pseudotumors appeared avascular on all images.

 


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Figure 7.  Photograph shows the gross surgical specimen with a partially exposed proximal pseudotumor.

 





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