Intra-extradural plexiform schwannoma of the cervical spine

Case report.

To describe the clinical and radiographic features of an extremely rare case with intra-extradural plexiform schwannoma of the cervical spine.

Plexiform schwannoma is a rare neurogenic tumor that predominantly occurs in the dermis and subcutis. Noncutaneous deep-seated lesions have rarely been described. No cases of intra-extradural plexiform schwannoma of the cervical spine have previously been reported.

A 16-year-old boy presented with a 3-month history of left neck and shoulder angle pain, motor weakness of the left upper extremity, clumsiness of bilateral hands, and mild gait disturbance. Preoperative magnetic resonance imaging showed a multinodular inhomogeneous dumbbell-shaped tumor encroaching on the cord at C3-C4. T1-weighted imaging showed the tumor as inhomogeneous with slightly higher intensity than muscle. T2-weighted imaging demonstrated a multinodular inhomogeneous tumor with much higher intensity than muscle, and each nodule of the tumor displayed a peripheral rim of higher intensity and central relatively lower intensity.

Through hemi-laminectomy from C3-C4 and facetectomy of the left side of C3-C4, the intradural parts of the multinodular tumor were completely extirpated and extradural parts of the multinodular tumor were enucleated as much as possible. Gross examination of the tumor showed yellowish-white soft contents that were encapsulated and multilobulated. Histologic examination revealed benign schwannoma. After surgery, preoperative neurologic symptoms completely resolved.

To the best of our knowledge, this is the first reported case of intra-extradural plexiform schwannoma. Careful magnetic resonance imaging might be helpful in suggesting this rare plexiform schwannoma before surgery.