Lipofibromatous Hamartoma of the Median Nerve: A Comprehensive Review and Systematic Approach to Evaluation, Diagnosis, and Treatment

Tahiri, Youssef; Xu, Liqin; Kanevsky, Jonathan; Luc, Mario

doi:10.1016/j.jhsa.2013.03.022

Cited by 64 publications

(60 citation statements)

References 116 publications

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“…Lipomatosis of nerve (LN) is a rare and incompletely understood benign condition, which has been variably described using terms such as fibrolipomatous hamartoma and fibrofatty proliferation of a nerve (43). LN most commonly occurs in the wrist and hand, involving the median nerve and its branches (43).…”

Section: Lipomatosis Of Nervementioning

confidence: 99%

Spectrum of Fat-containing Soft-Tissue Masses at MR Imaging: The Common, the Uncommon, the Characteristic, and the Sometimes Confusing

Gupta¹,

Potti²,

Wuertzer³

et al. 2016

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Fat-containing tumors are the most common soft-tissue tumors encountered clinically. The vast majority of fat-containing soft-tissue masses are benign. Lipomas are the most common benign fat-containing masses and demonstrate a characteristic appearance at magnetic resonance (MR) imaging. Less common benign soft-tissue masses include lipoblastoma, angiolipoma, spindle cell lipoma/pleomorphic lipoma, myolipoma, chondroid lipoma, lipomatosis of nerve, lipomatosis, hibernoma, and fat necrosis. Well-differentiated liposarcomas (WDLPSs)/atypical lipomatous tumors (ALTs) are locally aggressive soft-tissue masses that do not metastasize. Biologically more aggressive liposarcomas include myxoid, pleomorphic, and dedifferentiated liposarcomas. At MR imaging, lipomas typically resemble subcutaneous fat but may contain a few thin septa. The presence of thick, irregular, enhancing septa and nonfatty soft-tissue mass components suggests liposarcoma rather than lipoma. However, benign lipomatous lesions and WDLPS/ALT often have overlapping MR imaging findings. Distinguishing WDLPS/ALT from a benign lipomatous lesion or from fat necrosis at imaging can be challenging and often requires histologic evaluation. We present the spectrum of fat-containing masses, using the World Health Organization classification of adipocytic tumors, with an emphasis on commonly encountered lesions, characteristic MR imaging findings associated with specific tumors, and overlapping MR imaging findings of certain tumors that may require histologic sampling. We also briefly discuss the role of molecular markers in proper characterization and classification of fat-containing soft-tissue masses. (©)RSNA, 2016.

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Section: Lipomatosis Of Nervementioning

confidence: 99%

Spectrum of Fat-containing Soft-Tissue Masses at MR Imaging: The Common, the Uncommon, the Characteristic, and the Sometimes Confusing

Gupta¹,

Potti²,

Wuertzer³

et al. 2016

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“…However, MR imaging often shows an hourglass or fusiform enlargement with hypointense nerve bundles embedded in hyperintense adipose tissue. This gives a pathognomonic ‘spaghetti-like’ image in the sagittal plane and a ‘coaxial cable-like’ image in the axial plane 2 3. We managed our patient conservatively, as there are few therapeutic options.…”

mentioning

confidence: 95%

“…There is associated macrodactyly in 32% of cases 2. The clinical presentation is variable due location and digital hypertrophy.…”

mentioning

confidence: 99%

First digit macrodactyly and carpal tunnel syndrome caused by giant median nerve with macrodystrophia lipomatosa

et al. 2016

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A 63-year-old man reported numbness in his left first, second and third digits for 2 years. Initially, he found that 'shaking out' his left hand helped, but this benefit did not last. He underwent decompression of left-sided carpal tunnel syndrome 2 years earlier and his symptoms worsened after surgery, with more sensory loss and pain. He worked as a carpenter, and had injured his left thumb with nails on multiple occasions without feeling pain. On examination, he had an enlarged left thumb (figure 1), though he appeared unaware of this; his other digits were normal in size. The median nerve was palpable at the wrist, but Tinel's test was negative. There was a mild weakness of abductor pollicis brevis and opponens brevis, with reduced sensation over the fingertips of the lateral three digits and palm.Electrophysiological testing confirmed median nerve entrapment at the carpal tunnel, with absent sensory potentials at the third and fourth digits, with prolonged distal motor latency (5.6 ms), and decreased motor compound muscle action potential at the carpal tunnel (compound muscle action potential 2.7 mV). Proximal to the carpal tunnel inlet, the conduction velocities were normal (47.3 m/s at the forearm and 46.7 m/s at the upper arm). Electrodiagnostic testing of the ulnar nerve was normal. We did not perform needle electromyography. Ultrasound scan showed a hypertrophic enlarged median nerve along the entire tract in the left forearm (figure 2), but the main enlargement was at the wrist, where the cross-sectional area was 136 mm 2 (normal <12). There were no abnormalities in the ulnar and radial nerve, and plexus brachialis. A T1-weighted MR scan with gadolinium of the left arm showed an enlarged median nerve with lipomatous infiltration (figure 3), which is quite specific for macrodystrophia lipomatosa.The differential diagnosis of hypertrophied nerves has been discussed previously. 1 The pathophysiology that causes nerve hypertrophy can be very diverse, and includes entrapment neuropathies, leprosy, hereditary neuropathies and inflammatory neuropathies. Important sonographical features of nerve hypertrophy are diffuse or focal thickening and Figure 1 The patient's enlarged left thumb, with other digits of normal size.Figure 2 Ultrasound scan of the median nerve at the wrist, showing hypertrophic enlarged nerve with characteristic honeycomb structure.

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“…In the case of a large symptomatic tumor, neurolysis in addition to carpal tunnel release can be performed. 5 We present a case of lipofibromatous hamartoma of both the median and ulnar nerve at the level of the left wrist in a 30-year-old man with a palpable mass and hand pain. The presence of tumor involvement of both the median and ulnar nerves is rare and could result in nontraditional or confusing clinical manifestations involving both the median and ulnar nerve distribution.…”

mentioning

confidence: 99%

“…Carpal tunnel release can be performed in those who are asymptomatic but with a large tumor, or in effort to decrease motor and sensory impairments. In the case of a large symptomatic tumor, neurolysis in addition to carpal tunnel release can be performed …”

mentioning

confidence: 99%

Magnetic Resonance Imaging of Lipofibromatous Hamartoma Involving both the Median and Ulnar Nerve

Porrino

Wang

Branson

et al. 2019

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Lipofibromatous Hamartoma of the Median Nerve: A Comprehensive Review and Systematic Approach to Evaluation, Diagnosis, and Treatment

Cited by 64 publications

References 116 publications

Spectrum of Fat-containing Soft-Tissue Masses at MR Imaging: The Common, the Uncommon, the Characteristic, and the Sometimes Confusing

Spectrum of Fat-containing Soft-Tissue Masses at MR Imaging: The Common, the Uncommon, the Characteristic, and the Sometimes Confusing

First digit macrodactyly and carpal tunnel syndrome caused by giant median nerve with macrodystrophia lipomatosa

Magnetic Resonance Imaging of Lipofibromatous Hamartoma Involving both the Median and Ulnar Nerve

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