Fibrolipomatous hamartoma of the nerve

Assoc Prof Craig Hacking and Radswiki et al.

A fibrolipomatous hamartoma of the nerve (also known as a neural fibrolipoma or lipomatosis of the nerve) is a benign neoplasm of nerves, resulting from anomalous growth of fibroadipose tissue of the nerve sheath.

Fibrolipomatous hamartoma may be related to hypertrophy of mature fat and fibroblasts in the epineurium.

A fibrolipomatous hamartoma of the nerve can involve the median (80%), ulnar and or radial nerves and or dorsum of the hand.

The WHO classification of tumors of soft tissue categorizes this entity under adipocytic tumors 3.

Macrodactyly of the involved limb or digit can be present in ~ 66% of all cases of fibrolipomatous hamartoma of the median nerve.

A characteristic hypoechoic coaxial cabling encased by an echogenic substratum may be seen 3.

MRI features are often pathognomonic and typically shows a coaxial cable-like appearance on axial images and a spaghetti-like appearance on coronal images 2,6.

  • T1: the neural bundles were hyperintense to muscle and the surrounding substratum was isointense to muscle 3
  • T2: fat components are high signal and fibrous components are low signal

It is thought to have been first reported by M L Mason in 1953 5.

The lesion is benign and the preferred management of the lesion is conservative.

Article information

rID: 13422
Tag: cases, cases
Synonyms or Alternate Spellings:
  • Lipomatosis of nerve
  • Neural fibrolipoma
  • Lipofibromatous hamartoma of the nerve
  • Fibrolipomatous hamartoma of nerve

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Cases and figures

  • Case 1: classically affecting the median nerve
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  • Case 2: median nerve - with coaxial cable appearance
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  • Case 3: of the ulnar nerve
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