Neurofibromatosis type 2

Case contributed by Melbourne Uni Radiology Masters
Diagnosis certain


Back pain.

Patient Data

Age: 23-year-old
Gender: Female

Chest X-ray


Multiple right-sided mass lesions are seen consistent with history (on previous referrals) of neurofibromatosis. 5cm lesion arising from the right anterior chest wall is new. 5 x 3cm lesion projected over the right hilum, also arising from the anterior chest wall, is increased in size. 3.6cm right apical lesion is unchanged, with erosion of the posterior right 2nd rib. Small nodules projected over the mediastinum from T7-T9 could represent cutaneous lesions seen on previous MRI.

No focal consolidation. No pleural effusion or pneumothorax.

Left axillary surgical clips.

MRI Total spine


Right C1-C2: increase in nerve root sheath enhancing mass, previous 18 x 8 mm, current26 x 9 mm.

Cervicothoracic junction: 2 enhancing intra-axial lesions without cord compromise.Unchanged.Cervical intrathecal extra-axial enhancing lesions- C6 - a dumbbell lesion extending along C6 nerve root sheath: previous 10 x 11 mm,current 12 x 10 mm with appearances suggesting a slight increase in size and furtherdisplacement of the cord without cord signal change or enhancement. The nerve rootsheath extension is of similar appearance through the left foramen.

Right submandibular: previous 31 x 16 axial increasing to 38 x 21 mm with increase ofa mild degree to the subcutaneous nodules superior to this.Bilateral carotid sheath: unchanged.

Bilateral brachial plexus: unchanged noting the extension into the right lung apexregion.Right posterior triangle: unchanged.

Paraspinal muscles: slight increase in number and size.

Thoracic cord nodular enhancement: slight increase in thickness.

Thoracic intradural extra-axial nodules: slight increase in nodule size with dominantL1 nodule changing from April 11 22 x 12 mm, December 11 22 x 15 mm and current 26 x18 mm

Other sites: right intercostal, right subclavian, right paravertebral upper thoracic,subcutaneous and intercostal regions: slight increase in size - difficult to quantify.

Paraspinal enhancing nodule T5: previous 11 mm, current 12 mm.Syrinx T3-T4: unchanged.

Thoracolumbar region: cord expansion - known astrocytoma: unchanged.

Cauda equina - multiple enhancing nodules: unchanged.Lumbosacral plexus - plexiform neuroma: slight increase in size .Right parauterine enhancing lesion: previous 38 mm width,

Case Discussion

Prior imaging showed multiple lesions in keeping with NF 2

Remember the mnemonic "MISME": multiple inherited schwannomas, meningioma, and ependymoma. 

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