Fibrolipomatous Hamartoma of the Median Nerve

Clinical Cases 20.11.2006
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 30 years, male
Authors: Trueba R, Sarroca D, Buzzi A, Merola S, Rostagno C. Diagnostico Medico, Magnetic Resonance Department.
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AI Report

Clinical History

Soft tissue mass in the palm of the hand and carpal tunnel syndrome

Imaging Findings

A mechanic had a mass in the left palm that had been enlarging for several months. Decreased grip strength, numbness and tingling in the fingers had developed after minor trauma. The patient had no other clinical symptoms or history. We performed x rays, sonography and MRI with a GE Profile 0.2T. X rays showed a soft tissue mass in the palm. Sonography showed that the mass was in the middle of the carpal tunnel and had a cablelike appearance. MR images showed longitudinally orientated cylindrical areas of low signal intensity surrounded by adipose tissue in a diffusely thickened median nerve.

Discussion

Fibrolipomatous Hamartoma also known as lipomatosis of the median nerve is a rare benign lesion. More than 80% of these tumors are exclusively seen in the median nerve (1). It occurs most often at birth or infants and less commonly in children and young adults. There is no known cause or hereditary predisposition for this lesion, although hypertrophy of mature fat and fibroblasts in the epineurium has been postulated. The clinical manifestation is a slowly growing mass at the wrist, hand or forearm. Pain and neurological symptoms including carpal tunnel syndrome may be associated lately with lipomatosis of the median nerve. Macrodatyly is seen in 27% - 67% and has been referred to as macrodystrophia lipomatosa( 2, 3, 4). The MR appearance is unique and characteristic. The longitudinally orientated cylindrical region of signal void seen on all sequences represents the nerve fascicles and accompanying epineural and perineural fibrosis. These structures are separated by areas with signal intensity of fat, which represent the infiltrating mature fat cells in the interfascicular connective tissue (5). The differential diagnosis includes intraneural lipoma, ganglion cyst, traumatic neuroma and vascular malformations. Treatment includes carpal tunnel release. Surgical excision is controversial, motor and sensitive deficits have been reported (6,7).

Differential Diagnosis List

Fibrolipomatous Hamartoma of the Median Nerve  

Final Diagnosis

Fibrolipomatous Hamartoma of the Median Nerve  

Liscense

Figures

Hand picture and x rays

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Hand picture and x rays

Ultrasound

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Ultrasound

MRI

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MRI
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MRI
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MRI
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MRI