Desmoid tumor of the spinal canal causing scoliosis and paralysis.

Abstract

This report describes a case of successful surgical excision of an intrathoracic paraspinal desmoid tumor with an intraspinous extension causing scoliosis and paralysis in a 12-year-old girl. The purpose of this report is to illustrate the potential spectrum of disease of a desmoid tumor and to make physicians aware of the rare possibility of an intraspinal extension of a paraspinal desmoid tumor. Surgical excision of desmoid tumors in the pediatric population is the standard of care for initial treatment. There have been no clinical reports in the spine literature of a desmoid tumor causing scoliosis or of a desmoid tumor with an intraspinous extension causing paralysis. The tumor was decompressed from a posterior approach followed by an anterior resection and an anterior spinal fusion. Two weeks later a posterior spinal fusion was performed to correct the spinal deformity. The patient also received radiation therapy after surgery for 5 weeks. The patient tolerated the procedure well, has been free of recurrence for 9 years, and is currently doing well. This case report should help expand the understanding of the spectrum of this uncommon tumor.