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MRI and Biologic Behavior of Desmoid Tumors in Children

M. Beth McCarville1,2, Fredric A. Hoffer1,2, C. Scott Adelman1, Joseph D. Khoury3, Chenghong Li4 and Stephen X. Skapek5,6

1 Division of Diagnostic Imaging, Department of Radiological Sciences, St. Jude Children's Research Hospital, 332 N Lauderdale St., Memphis, TN 38105-2794.
2 Department of Radiology, University of Tennessee, College of Medicine, Memphis, TN.
3 Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN.
4 Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN.
5 Department of Hematology–Oncology, University of Tennessee, College of Medicine, Memphis, TN.
6 Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN.


Figure 1
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  		Fig. 1A —19-year-old male with Gardner syndrome and massive desmoid tumor of paraspinal musculature. Baseline sagittal T2-weighted MR image (TR/TE, 5,950/116) shows hypointense bands (arrows) coursing through hyperintense tumor.

 

Figure 2
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  		Fig. 1B —19-year-old male with Gardner syndrome and massive desmoid tumor of paraspinal musculature. Sagittal unenhanced T1-weighted MR image (618/14, B) and contrast-enhanced T1-weighted image (804/14, C) show lack of enhancement of hypointense bands (arrows) in A. These nonenhancing, hypointense bands are probably areas of fibrosis within enhancing active fibroblasts. Patients with Gardner syndrome or familial adenomatous polyposis have 20% lifetime risk of extraabdominal or, more commonly, intraabdominal desmoid tumor.

 

Figure 3
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  		Fig. 1C —19-year-old male with Gardner syndrome and massive desmoid tumor of paraspinal musculature. Sagittal unenhanced T1-weighted MR image (618/14, B) and contrast-enhanced T1-weighted image (804/14, C) show lack of enhancement of hypointense bands (arrows) in A. These nonenhancing, hypointense bands are probably areas of fibrosis within enhancing active fibroblasts. Patients with Gardner syndrome or familial adenomatous polyposis have 20% lifetime risk of extraabdominal or, more commonly, intraabdominal desmoid tumor.

 

Figure 4
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  		Fig. 2A —10-month-old girl with desmoid tumor of middle finger. T2-weighted coronal image (TR/TE, 3,000/30) shows predominantly hyperintense mass (arrows) with fairly sharp margins, except for proximal and distal margins.

 

Figure 5
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  		Fig. 2B —10-month-old girl with desmoid tumor of middle finger. T1 contrast-enhanced coronal image (400/20) shows peripheral tumor enhancement and central lack of enhancement (arrows). Although small, this tumor necessitated amputation of digit for local control.

 

Figure 6
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  		Fig. 3A —14-year-old girl with recurrent desmoid tumor in left foot. Sagittal STIR image (TR/TE, 2,500/18; flip angle, 140°) 2 weeks after completion of radiation therapy shows tumor (arrows) predominantly hyperintense to muscle. Some of hyperintense signal may be result of edema from recent radiation.

 

Figure 7
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  		Fig. 3B —14-year-old girl with recurrent desmoid tumor in left foot. Sagittal STIR image (3,500/18; flip angle, 140°) 2 years 7 months after A shows tumor (arrows) has grown slightly but is predominantly hypointense to muscle.

 

Figure 8
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  		Fig. 4A —2-year-old girl with desmoid tumor in right lateral aspect of chest. T2-weighted axial image (TR/TE, 4,000/90) shows uniformly hypointense tumor (arrows) in right chest wall muscles.

 

Figure 9
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  		Fig. 4B —2-year-old girl with desmoid tumor in right lateral aspect of chest. Unenhanced T1-weighted axial image (25/9, B) and contrast-enhanced T1-weighted axial image (765/14, C) show no enhancement of tumor (arrows) at this level.

 

Figure 10
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  		Fig. 4C —2-year-old girl with desmoid tumor in right lateral aspect of chest. Unenhanced T1-weighted axial image (25/9, B) and contrast-enhanced T1-weighted axial image (765/14, C) show no enhancement of tumor (arrows) at this level.

 

Figure 11
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  		Fig. 4D —2-year-old girl with desmoid tumor in right lateral aspect of chest. Photomicrograph of biopsy specimen obtained 5 days after A–C reveals sparse cells (5%) separated by prominent collagen bundles. Entrapped peripheral nerve (arrows) is evident.

 

Figure 12
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  		Fig. 5A —14-year-old girl with desmoid tumor in left ankle. Findings contrast to MRI features in Figures 4A, 4B, 4C, and 4D. STIR coronal image (TR/TE, 3,500/18; flip angle, 90°) shows tumor has uniformly high signal intensity (arrows) relative to muscle.

 

Figure 13
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  		Fig. 5B —14-year-old girl with desmoid tumor in left ankle. Findings contrast to MRI features in Figures 4A, 4B, 4C, and 4D. Unenhanced T1-weighted axial image (650/15, B) and contrast-enhanced T1-weighted axial image (710/15, C) show uniform moderate tumor enhancement (arrows).

 

Figure 14
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  		Fig. 5C —14-year-old girl with desmoid tumor in left ankle. Findings contrast to MRI features in Figures 4A, 4B, 4C, and 4D. Unenhanced T1-weighted axial image (650/15, B) and contrast-enhanced T1-weighted axial image (710/15, C) show uniform moderate tumor enhancement (arrows).

 

Figure 15
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  		Fig. 5D —14-year-old girl with desmoid tumor in left ankle. Findings contrast to MRI features in Figures 4A, 4B, 4C, and 4D. Photomicrograph of biopsy specimen obtained 2 weeks after A–C shows 50% cellularity with rare collagen bands.

 

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