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Lipofibromatous Hamartoma of the Upper Extremity: A Review of the Radiologic Findings for 15 Patients

Andoni P. Toms1, Dimitri Anastakis2, Robert R. Bleakney3 and Thomas J. Marshall

1 Department of Radiology, Norfolk and Norwich University Hospital, Colney Ln., Norwich, Norfolk NR4 7UY, England.
2 Toronto Western Hospital, University Health Network, Toronto, ON, Canada.
3 Mount Sinai Hospital, University Health Network, Toronto, ON, Canada.


Figure 1
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  		Fig. 1 —52-year-old man with lipofibromatous hamartoma. Intraoperative photograph illustrates typical gross appearance of lipofibromatous hamartoma. Multiple soft, gray-yellow lobulated masses (arrows) are present within epineural sheath of radial digital nerve of this right index finger. Epineurium of digital nerve had extensive perineural fibrosis.

 

Figure 2
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  		Fig. 2A —52-year-old man with lipofibromatous hamartoma of median nerve and macrodystrophia lipomatosa. Photograph of volar aspect of right hand and forearm shows local gigantism of thumb and radial half of index finger (arrowheads), with associated peripheral cyanosis. Scar (arrow) extending from thenar eminence proximally into forearm bears witness to previous surgical exploration and decompression of "fatty mass."

 

Figure 3
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  		Fig. 2B —52-year-old man with lipofibromatous hamartoma of median nerve and macrodystrophia lipomatosa. Conventional radiograph of same patient shows regional osseous gigantism in median nerve distribution. Premature osteoarthrosis of interphalangeal joints (arrowheads) has been treated by arthrodesis and internally fixed with cerclage wires.

 

Figure 4
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  		Fig. 3 —31-year-old woman who presented with paresthesia in ulnar nerve distribution. Axial T1-weighted MR image through wrist at level of distal carpal row shows Guyon's canal distended with fat (arrowhead) and thickened fascicles of ulnar nerve. Conductive delay was confirmed with electromyelography.

 

Figure 5
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  		Fig. 4 —41-year-old man with lipofibromatous hamartoma of median nerve. Coronal T1-weighted MR image of hand shows that tumor (arrow) is predominantly lipomatous and extends from Guyon's canal. Palmar mass had first been noticed at age of 6 years and grew slowly for 35 years until presentation.

 

Figure 6
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  		Fig. 5 —40-year-old woman with lipofibromatous hamartoma of median nerve. Axial T1-weighted MR image of wrist at level of proximal carpal row shows that median nerve is enlarged, as are individual neural bundles, but that signal characteristics are those of normal nerve. Neural bundles have higher T1 signal (arrow) than does normal muscle, and no discrete lipomatous component is present at this level.

 

Figure 7
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  		Fig. 6A —31-year-old woman with extensive lipofibromatous hamartoma of upper limb nerves. Sonograms of enlarged median nerve acquired in sagittal (A) and axial (B) planes show hypoechoic cablelike neural bundles (arrows) separated by hyperechoic fat.

 

Figure 8
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  		Fig. 6B —31-year-old woman with extensive lipofibromatous hamartoma of upper limb nerves. Sonograms of enlarged median nerve acquired in sagittal (A) and axial (B) planes show hypoechoic cablelike neural bundles (arrows) separated by hyperechoic fat.

 

Figure 9
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  		Fig. 7A —55-year-old man with lipofibromatous hamartoma of median nerve. Coronal T1-weighted MR image shows lipofibromatous hamartoma of brachial plexus (arrow) at level of formation of terminal branches.

 

Figure 10
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  		Fig. 7B —55-year-old man with lipofibromatous hamartoma of median nerve. Axial T1-weighted image through arm shows lipofibromatous hamartoma of radial and median nerves (arrows).

 

Figure 11
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  		Fig. 8 —31-year-old man. Axial T1-weighted MR image through wrist shows enlarged median nerve and divided flexor retinaculum. Signal intensities of nerve are reversed from typical pattern seen in lipofibromatous hamartoma, with neural fascicles of high fat intensity (arrow) and intervening substratum of intermediate soft-tissue intensity.

 

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