Cervical spinal neurofibroma in a patient with NF1

Case contributed by Dr Maxime St-Amant

Presentation

Increased neck pain; previous history of the patient hidden for the purpose of this case.

Patient Data

Age: 40
Gender: Male

There is a large solid intraspinal and extraspinal mass centered at the left C4/C5 foramen also extending in the left C5/C6 foramen. It extends cephalocaudally from C2 to C6. It causes marked rightward displacement of the cervical cord as it extends within the spinal canal and occupies more than 50% of the surface. There is scalloping of the adjacent bone, suggesting a longstanding mass. There are peripheral calcifications in its anterior aspect, most likely due to bone remodelling (transverse foramen) that actual tumoral calcifications.

There is loss of the normal cervical lordosis with scalloping of posterior vertebral bodies below the tumor mass, suggesting the possibility of dural ectasia.

The previously described intraspinal and extraspinal mass appears heterogeneous on T1/T2. It is mainly T1 hyperintense and markedly T2 hypointense with susceptibility artefacts, suggesting the presence of blood products. The presence of internal blood products is also demonstrated on GRE. There is heterogeneous enhancement of the mass but intrinsic T1 hyperintense signal makes this more difficult to assess.

There is marked mass effect/compression of the cervical cord with rightward deviation.

There is proximal occlusion of the proximal left vertebral artery.

The examination confirms dural ectasia. 

Case Discussion

This patient has a previous history of NF1 as suggested by the presence of dural ectasia. The most likely diagnosis for such a lesion is a neurofibroma considering the previous history and radiological findings. However, hemorrhagic changes are atypical. Internal calcifications are ever rarer.

The main differentials would be schwannoma and meningioma.

Pathology report:

  • Dense fibrous tissue, pieces of old altered blood and one piece of tumor tissue.
  • The tumor tissue is moderately cellular and consists of spindle cells with small, bland nuclei and inconspicuous cytoplasm, within a dense fibrous stroma.  The cells are haphazardly arranged.
  • No mitoses are identified and there is no tumor necrosis.
  • The tumor cells stain S100 positive and the features are most consistent with a neurofibroma.
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Case information

rID: 58344
Published: 13th Feb 2018
Last edited: 16th Jul 2018
System: Spine
Inclusion in quiz mode: Included

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