CASE 17

By: Dr. Sattam Lingawi and Dr. Alaa Al-Turkustani

HISTORY:  Withheld.

What are your findings?

What is the differential diagnosis?

FINDINGS:

Fig. 1 sagittal T2 WI and Fig. 2 sagittal post contrast T1 WI with fat saturation of the cervical spine demonstrating multiple intra-medulary T2 bright signal intensity masses involving the cervical and upper thoracic cord at the level of C2/3, C4, C7/T1, T2/3 and T3/4 with the largest mass at C4 level causing cord expansion and demonstrate avid enhancement post contrast administration.

Fig. 3 sagittal T2 WI and Fig. 4 sagittal post contrast T1 WI with fat saturation the lumbar spine demonstrating multiple enhancing nodules within the conus medullaris and filum terminale likely representing drop metastases.

DIFFERENTIAL DIAGNOSIS:

• Intramedullary Ependemoma or astrocytoma with drop metastasis.

• Spinal metastasis.

DIAGNOSIS:

Intra medullary ependemomas with drop metastasis in patient with neurofibromatosis type 2.

PEARLS AND DISCUSSION:

Fig. 5 and Fig. 6 axial post contrast T1 WI of the brain of the same patient demonstrating multiple parasagittal and left orbital meningiomata and bilateral vestibular schwannomas.

Fig. 7 and Fig. 8 sagittal and coronal T2 WI with fat saturation, Fig. 9 sagittal T1 WI and Fig. 10 sagittal T1 WI with fat saturation demonstrating T2 bright signal intensity lesion in the right apical area with strong enhancement post contrast administration possibly representing schwannoma.

Neurofibromatosis type 2 (NF2) is an inherited autosomal dominant syndrome characterized by multiple schwannomas, meningiomas, and ependymomas.  The most common tumor associated with the syndrome is the vestibulocochlear (cranial nerve [CN] VIII) schwannoma, and as many as 10% of patients with this tumor have NF2.

It is not associated with neurofibromas. Instead, patients with this disease have:

• Intracranial schwannoma(s): mostly vestibular schwannoma(s).

• Intracranial and spinal meningioma(s).

• Intraspinal-intramedullary ependymoma(s).

FURTHER READING:

1. Andrew L Wagner, Neurofibromatosis Type 2 Imaging. Rockingham Memorial Hospital

2. Kiran Gangadhar, Sandeep Kumar. A Complete Constellation of Nervous System Lesions of NF2: Imaging Evaluation. Case Reports in Radiology 353179

3. LubdhaM. Shah andKaren L. Salzman, Imaging of Spinal Metastatic Disease, Hindawi Publishing Corporation International Journal of Surgical Oncology Volume 2011, Article ID 769753, 12 pages doi:10.1155/2011/769753

4. Jin Kyeong Sung, MD, Won-Hee Jee, MD, Differentiation of acute Osteoporotic and Malignant Compression Fractures of the spine: Use of Additive Qualitative and Quantitative Axial Diffusion-weighted MR Imaging to Conventional MR Imaging at 3.0T