Presentation
Known from clinics due to previous spinal lesion resection.
Patient Data
There are multiple enhancing intracanal and neural foraminal lesions, the largest involves the right T4 nerve root, measuring 3.4 cm in A-P diameter, demonstrating heterogeneous enhancement with central low signal suggesting necrosis. There is also a large paraspinal lesion at T8-T10, extending along the anterior surface of the vertebral bodies over a vertical length of approximately 5 cm.
Several lesions related to both posterior ribs, subcutaneous lesions, and also a large one at the base of the tongue.
Case Discussion
The imaging features are those of multiple well-defined enhancing masses throughout the spinal canal, intervertebral foramina, and a few along the imaged soft tissues. Appearances overall favour multiple nerve sheath tumours. The spinal lesions are intradural-extramedullary. Small spinal meningiomas can not be ruled out.
This patient is known from clinics given its neurofibromatosis type II (NF2) diagnosis, which a prominent spinal involvement. He also has a tentorial likely meningioma and bilateral acoustic schwannomas (not shown). The appearances of the spine alone cannot distinguish between NF 1 and NF 2.
NF2 is one of the phakomatosis syndromes and is characterised by mutations in the chromosome 22q12, affecting the gene responsible for the production of protein merlin, a tumour suppressor protein.
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