Spinal intramedullary epidermoid cysts: Three case presentations and literature review

Abstract

Background: True intramedullary epidermoid cysts (IECs) not associated with congenital anomalies or previous spinal procedures are extremely rare. In a review of the literature since 1992, only 29 such cases have been reported. Here, we add three new cases in this category.

Case description: Three adults presented with spastic paraparesis attributed to thoracic IECs. Gross total microsurgical removal was achieved in two cases, while one case was a partial resection due to capsular adherence to the cord. In all three cases, patients sustained complete recoveries of neurological function and remained symptom free for an average of 5 years follow-up.

Conclusion: IECs are rare lesions; here, the three located in the thoracic spine, contributed to slow, progressive spastic paraparesis with/without incontinence, and resolved following total (2 patients) and partial (1 patient) resection.

Keywords: Epidermoid cyst; Intramedullary tumor; Paraparesis; Spinal cord; Thoracic spine.

Figure 1:

(a) Cervicothoracic T1-weighted magnetic resonance imaging (MRI) a hypointense tumor at T3-T4 level, (b) in T2-weighted MRI the mass is hyperintense, (c) intraoperative photograph; a white avascular tumor inside the cord, (d) after total removal, (e) postoperative T2-weighted MRI which is clear from residue.

Figure 2:

(a) Cervicothoracic T1-weighted sagittal magnetic resonance imaging (MRI) shows a mixed hypo-hyperintense mass at T3-T4 level, (b) in T2-weighted image, the mass is also hypo-hyperintense which is not usual in intramedullary epidermoid cysts, (c) but in fat-suppressed MRI the whole of the tumor is hyperintense, (d) intraoperative photograph; a milky white tumor is demonstrated after midline myelotomy, (e) after total removal of the mass.

Figure 3:

(a) Cervicothoracic T1-weighted magnetic resonance imaging (MRI) the tumor is isointense, (b) in T2-weighted mage it is hypointense which is unusual for intramedullary epidermoid cysts, (c) but in fat-suppressed MRI the hyperintense mass is compatible with epidermoid, (d) intraoperative photograph shows the characteristic features of and epidermoid cyst, (e) after total removal, (f) T1-weighted MRI at 10-year follow-up shows neither residue nor recurrence, (g) T2-weighted image also is clear.