J Korean Orthop Assoc.  2012 Apr;47(2):160-163. 10.4055/jkoa.2012.47.2.160.

Thoracolumbar Intradural Schwannoma in a Patient with Osteopetrosis

• Goh TS
• Lim JM
• Lee JS

• KMID: 2185394
• DOI: http://doi.org/10.4055/jkoa.2012.47.2.160

Abstract

Schwannoma is a benign tumor derived from Schwann cells which are usually found in the head, neck, trunk, upper and lower extremities, and rarely in the thoracolumbar junction. Also, osteopetrosis is an idiopathic genetic skeletal disease that rarely occurs. Most reports of osteopetrosis are about long bone fractures, but spinal tumor in osteopetrosis has been rarely reported. Furthermore, there is no report about thoracolumbar intradural Schwannoma and its treatment in patient with osteopetrosis. Thus, we report the clinical results of a thoracolumbar Schwannoma in a 36 year-old female osteopetrotic patient who had progressive weakness, and dysesthesia of the left lower limb. The patient underwent laminectomy and total excision of tumor through a posterior approach, and a favorable clinical outcome was achieved.

Keyword

Osteopetrosis; Schwannoma

MeSH Terms

Female
Fractures, Bone
Head
Humans
Laminectomy
Lower Extremity
Neck
Neurilemmoma
Osteopetrosis
Paresthesia
Schwann Cells

Figure

• 

  Figure 1 (A) Initial thoracolumbar spine anteroposterior, and (B) lateral radiographs showing diffuse, extensive and homogeneous osteopetrosis.

• 

  Figure 2 (A) T1 weighted, (B) T2 weighted, and (C) enhanced images demonstrate diffuse dark signal patterns of the entire skeleton due to osteopetrosis and extramedullary neurogenic tumor on left side of T12/L1 level.

• 

  Figure 3 Intraoperative photograph shows well encapsulated yellowish intradural mass.

• 

  Figure 4 Spindle tumor cells show palisaded arrangement (H & E stain, ×400).

Reference

1. Abernathey CD, Onofrio BM, Scheithauer B, Pairolero PC, Shives TC. Surgical management of giant sacral schwannomas. J Neurosurg. 1986. 65:286–295.

2. Albers-Schonberg HE. Rontgenbilder einer seltenen Knochenerkrankung. Munchen Med Wchnschr. 1904. 51:365.

3. Chhabra A, Westerlund LE, Kline AJ, McLaughlin R. Management of proximal femoral shaft fractures in osteopetrosis: a case series using internal fixation. Orthopedics. 2005. 28:587–592.

4. Milgram JW, Jasty M. Osteopetrosis. A morphological study of twenty-one cases. J Bone Joint Surg Am. 1982. 64:912–929.

5. Montgomery RD, Standard KL. Albers-Schoenberg's disease. A changing concept. J Bone Joint Surg Br. 1960. 42-B:303–312.

6. Kim P, Ebersold MJ, Onofrio BM, Quast LM. Surgery of spinal nerve schwannoma. Risk of neurological deficit after resection of involved root. J Neurosurg. 1989. 71:810–814.

7. Acciarri N, Staffa G, Poppi M. Giant sacral schwannoma: removal by an anterior, transabdominal approach. Br J Neurosurg. 1996. 10:489–492.

8. Turk PS, Peters N, Libbey NP, Wanebo HJ. Diagnosis and management of giant intrasacral schwannoma. Cancer. 1992. 70:2650–2657.