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Imaging Appearance of Diffuse Neurofibroma

Douglass S. Hassell1, Laura W. Bancroft1, Mark J. Kransdorf1,2, Jeffrey J. Peterson1, Thomas H. Berquist1, Mark D. Murphey2,3 and Julie C. Fanburg-Smith4

1 Department of Radiology, Mayo Clinic, 4500 San Pablo Rd., Jacksonville, FL 32224-3899.
2 Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC.
3 Departments of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD.
4 Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC.


Figure 1
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  		Fig. 1A —31-year-old man with neurofibromatosis. Histologic features of diffuse neurofibroma. Photomicrograph shows diffuse neurofibroma cells with short fusiform and round shapes present within fine fibrillary collagen background (black arrows). Scattered fat cells (white arrows) are present.

 

Figure 2
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  		Fig. 1B —31-year-old man with neurofibromatosis. Histologic features of diffuse neurofibroma. Photomicrograph shows multiple laminated bodies (arrows) that resemble Wagner-Meissner tactile corpuscles and are characteristic of diffuse neurofibroma.

 

Figure 3
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  		Fig. 2A —65-year-old man with plaquelike diffuse neurofibroma. Sagittal T1-weighted MR image (TR/TE, 500/16) shows thick plaquelike diffuse neurofibroma involving skin and subcutaneous tissues of back. Deep aspect (black arrow) of mass is well-defined, and deeper subcutaneous tissues are uninvolved. Small flow voids (white arrows) reflect prominent internal vascularity.

 

Figure 4
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  		Fig. 2B —65-year-old man with plaquelike diffuse neurofibroma. Sagittal T2-weighted MR image (3,200/104) shows diffuse neurofibroma (white arrows) is markedly hyperintense in relation to muscle (black arrow).

 

Figure 5
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  		Fig. 3A —24-year-old man with plaquelike diffuse neurofibroma of left temporal region with intraorbital extension. Axial T1-weighted MR image (TR/TE, 350/16) shows plaquelike diffuse neurofibroma extending from left temporal (thick arrow) to nasal region. Mass extends into orbit (thin arrow).

 

Figure 6
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  		Fig. 3B —24-year-old man with plaquelike diffuse neurofibroma of left temporal region with intraorbital extension. Axial T2-weighted MR image (2,926/80) shows diffuse neurofibroma (arrows) to be mildly hyperintense in relation to muscle but of overall intermediate signal intensity.

 

Figure 7
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  		Fig. 3C —24-year-old man with plaquelike diffuse neurofibroma of left temporal region with intraorbital extension. Axial contrast-enhanced T1-weighted MR image (400/19) shows prominent enhancement of diffuse neurofibroma (arrows).

 

Figure 8
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  		Fig. 4A —31-year-old woman with diffuse neurofibroma of left lateral buttock region shows infiltrative growth pattern. Axial T1-weighted MR image (TR/TE, 650/11) shows infiltrative diffuse neurofibroma (arrow) centered within subcutaneous tissue of left lateral buttock region.

 

Figure 9
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  		Fig. 4B —31-year-old woman with diffuse neurofibroma of left lateral buttock region shows infiltrative growth pattern. Axial contrast-enhanced T1-weighted MR image (550/12) with fat saturation shows enhancement of infiltrative diffuse neurofibroma with extension to skin (thick arrow) and fascia (thin arrow) overlying right gluteal musculature.

 

Figure 10
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  		Fig. 5A —20-year-old man with masslike diffuse neurofibroma of right frontal region. Axial fat-suppressed T2-weighted MR image (TR/TE, 2,200/80) shows diffuse neurofibroma (arrow) to be mildly hyperintense in relation to skeletal muscle (seen on other axial images) or of overall intermediate signal intensity.

 

Figure 11
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  		Fig. 5B —20-year-old man with masslike diffuse neurofibroma of right frontal region. Coronal contrast-enhanced T1-weighted MR image (500/17) with fat saturation shows intense enhancement of diffuse neurofibroma (arrow).

 

Figure 12
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  		Fig. 6A —62-year-old woman with diffuse neurofibroma of right buttock. Axial T1-weighted MR image (TR/TE, 467/9) shows plaquelike thickening of skin (thin white arrow) of right gluteal region, which is slightly hyperintense in relation to muscle. At deep aspect of skin thickening, diffuse neurofibroma becomes more infiltrative (thick white arrow). Diffuse neurofibroma extends to and infiltrates right gluteus maximus muscle (black arrow), which is markedly enlarged.

 

Figure 13
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  		Fig. 6B —62-year-old woman with diffuse neurofibroma of right buttock. Axial contrast-enhanced T1-weighted MR image (594/10) with fat saturation shows diffuse infiltration and marked enlargement of gluteus maximus muscle (arrow) with maintenance of overall muscle structure.

 

Figure 14
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  		Fig. 6C —62-year-old woman with diffuse neurofibroma of right buttock. Sonographic image obtained during biopsy of diffuse neurofibroma shows mixed sonographic appearance. Superficial subcutaneous portion (black arrow) of mass is hyperechoic with small irregular hypoechoic areas. Deep intramuscular portion (white arrow) of mass is hypoechoic.

 

Figure 15
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  		Fig. 7 —Chart shows distribution of 349 diffuse neurofibromas in 339 patients.

 

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