Pancreatic neurofibroma

Last revised by Jeremy Jones on 20 Sep 2021

Pancreatic neurofibromas are rare nonepithelial neoplasms of the pancreas. They are similar to neurofibromas found elsewhere in the body, and are associated with neurofibromatosis type I

If they do cause symptoms, it will typically be those related to regional mass effect.

Neurofibromas arise from the elements of peripheral nerves, including Schwann cells.

Histologically, benign spindle cells are present in a myxoid fibrillary matrix. Neurofibromas express S100, but no P53 and limited Ki-67.

  • well-circumscribed
  • hypoattenuating
  • mildly enhancing
  • T1: hypointense
  • T2: hyperintense

Classic appearance is a "target" with a T2 hypointense core surrounded by a T2 hyperintense ring, similar to neurofibromas elsewhere in the body. The myxoid component of the tumor results in the T2 hyperintensity.

  • cystic change, calcification, and hemorrhage may be present
  • well-defined
  • hypoechoic with posterior through-transmission

Malignant transformation has been estimated to occur in 7-13% of neurofibromas 2. Because of its location, neurofibromas in the pancreas may be treated conservatively.

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