In those without neurofibromatosis type 2 (NF2), they tend to present between the 3rd to 6th decades of life. There is a recognised female predilection 1. The tumours can have a wide variety of clinical presentations.
They can arise from cranial nerves IX, X or XI, with IX being the most common 3.
- neurofibromatosis type 2 (NF2): particularly if bilateral
- usually well demarcated
- tend to be iso to hypoattenuating to brain parenchyma 3
- may show expansion and remodelling of the affected jugular foramen
- may have a characteristic dumbbell configuration
Signal characteristics are those of a schwannoma:
- T1: typically low signal
T2: typically high signal
- cystic degeneration may be seen in larger tumours
T1 C+ (Gd)
- smaller lesions demonstrate intense homogeneous enhancement
- larger lesions tend to have heterogeneous enhancement
Treatment and prognosis
As with most schwannomas, they tend to be slow growing tumours. Surgical resection is often the treatment of choice.
For a full list of differentials see the article on jugular fossa masses. Considerations in this location include:
- 1. Ortiz O, Reed L. Spinal accessory nerve schwannoma involving the jugular foramen. AJNR Am J Neuroradiol. 1995;16 (4): 986-9. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 2. Macdonald AJ, Salzman KL, Harnsberger HR et-al. Primary jugular foramen meningioma: imaging appearance and differentiating features. AJR Am J Roentgenol. 2004;182 (2): 373-7. AJR Am J Roentgenol (full text) - Pubmed citation
- 3. Eldevik OP, Gabrielsen TO, Jacobsen EA. Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol. 21 (6): 1139-44. AJNR Am J Neuroradiol (full text) - Pubmed citation