Pancreatic neurofibroma
Last revised by Jeremy Jones on 20 Sep 2021
Citation, DOI, disclosures and article data
Citation:
Morgan M, Weerakkody Y, Pancreatic neurofibroma. Reference article, Radiopaedia.org (Accessed on 20 Apr 2024) https://doi.org/10.53347/rID-42908
Permalink:
rID:
42908
Article created:
14 Feb 2016,
Matt A. Morgan ◉
Disclosures:
At the time the article was created Matt A. Morgan had no recorded disclosures.
View Matt A. Morgan's current disclosures
Last revised:
20 Sep 2021,
Jeremy Jones ◉
Disclosures:
At the time the article was last revised Jeremy Jones had no recorded disclosures.
View Jeremy Jones's current disclosures
Revisions:
2 times, by
2 contributors -
see full revision history and disclosures
Systems:
Synonyms:
- Pancreatic neurofibromas
- Pancreatic neurofibromata
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.
On this page:
Clinical presentation
If they do cause symptoms, it will typically be those related to regional mass effect.
Pathology
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.
Radiographic features
CT
- well-circumscribed
- hypoattenuating
- mildly enhancing
MRI
- 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
US
- well-defined
- hypoechoic with posterior through-transmission
Treatment and prognosis
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.
Differential diagnosis
-
pancreatic neuroendocrine tumor
- more common overall
- may have a syndromic presentation
-
pancreatic Schwannoma:
- associated with neurofibromatosis type 2
- will have a different histological appearance
- a mucinous lesion of the pancreas with features suspicious for malignancy
- will have characteristic findings on EUS biochemistry and cytology
References
- 1. Manning MA, Srivastava A, Paal EE et-al. Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1-Benign Tumors: From the Radiologic Pathology Archives. Radiographics. 2016;36 (1): 123-41. doi:10.1148/rg.2016150212 - Pubmed citation
- 2. Delgado J, Jaramillo D, Ho-Fung V et-al. MRI features of plexiform neurofibromas involving the liver and pancreas in children with neurofibromatosis type 1. Clin Radiol. 2014;69 (6): e280-4. doi:10.1016/j.crad.2014.01.020 - Pubmed citation
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