Neurofibromatosis with brachial plexus neurofibroma

Case contributed by Dr Shu Su

Presentation

41yo male with history of neurofibromatosis and multiple neurogenic tumours. MRI monitoring of largest lesion in right supraclavicular fossa and right brachial plexus.

Patient Data

Age: 41
Gender: Male

Innumerable enhancing neurogenic lesions are identified.  The largest lesion in the right supraclavicular fossa (that appears to be related to the right C6 nerve root) measures 43 x 52 mm, unchanged. Indentation of the cervical cord by neurogenic tumours arising from the right C6 nerve root and left C4 nerve root is unchanged. No cord signal abnormality. Numerous neurogenic tumours are seen scattered along the cervical cord, involving most neural exit foramen.

Case Discussion

There are three main types of neurogenic tumours formed directly from the brachial plexus – benign schwannomas, benign neurofibromas and malignant peripheral nerve sheath tumour (MPNST).

One-third of neurofibromas are associated with neurofibromatosis. It is important to distinguish neurofibromas as solitary lesions can often be removed without nerve damage 1

Although it may be difficult to distinguish between neurofibromas and schwannomas on MRI, some of the more typical MRI features of neurofibroma are listed below 1-4:

  • architecture
    • fusiform distension of the nerve with diffuse loosening of the nerve fibre
    • no capsule 
    • nerve enters and leaves through the centre of the tumour 
    • no peritumoural oedema
  • signal intensity
    • similar to muscle on T1-weighted images, marked increased intensity on T2-weighted images 
    • target sign (as demonstrated in this case) – hyperintense rim of myxoid tissue with central hypointense area of fibrocollagenous tissue on T2-weighted imaging – appears in 58% of neurofibromas and 15% of schwannomas
  • contrast
    • central homogenous enhancement with gadolinium contrast 
  • associated features
    • can have mild muscle atrophy distal to tumour 

Case courtesy of A/Prof Pramit Phal.

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Case Information

rID: 31962
Case created: 8th Nov 2014
Last edited: 30th Sep 2015
Inclusion in quiz mode: Included

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