Lipofibromatous hamartoma

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[edit] Discussion of Lipofibromatous hamartoma

  • Proliferation of mature adipocytes within peripheral nerves resulting in a palpable yellow neurogenic mass.
  • Mature fat infiltrates the nerve, separating axonal bundles sheathed in perineurium, which normally lie adjacent to each other.
  • Lipofibromatous hamartomas are rare.
  • Patients typically present in their third or fourth decade of life with a long history of painless swelling in the distal forearm that often was first noted in childhood.
  • The median nerve is the most commonly involved peripheral nerve, with involvement of other peripheral nerves of the upper extremity reported less frequently.
  • Patients with median nerve involvement commonly present with carpal tunnel syndrome.
  • Before the widespread availability of MRI, the diagnosis of lipofibromatous hamartoma was confirmed after exploratory surgery and biopsy of the lesion.

[edit] Imaging Findings for Lipofibromatous hamartoma

  • The pathognomonic pattern is an enlarged nerve containing 15 or 16 coaxial "cables" or bundles of axons encased in epineural fibrous tissue.
  • In the region of the carpal tunnel, the neural bundles were evenly interspersed with fat, such that the cross-sectional area of the median nerve is increased to 0.6-1.6 cm2.
  • Sonography showed the characteristic hypoechoic coaxial cabling encased by an echogenic substratum.

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Lipofibromatous hamartoma