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An Unusual Primary Osseous Tumor: Desmoplastic Fibroma of the Thoracic Spine 2014

Category General Spine Paul A. Hill, M.D., M.S.
David A. Pastel, MD
Purpose To report a rare case of desmoplastic fibroma of the thoracic spine causing significant myelopathic symptoms. Materials & Methods The clinical documents and relevant imaging was reviewed. Results A 46 year old male with a five year history of lower back pain and prior L5-S1 discectomy presented with new onset of parasthesias, myelopathic gait and hyperreflexia. A total spine MR revealed a homogenous T1 & T2 hypointense expansile mass involving the T3 vertebral body, spinous process and articulating processes with epidural extension causing cord compression. CT examination detected a destructive lesion with a large lytic defect in the T3 vertebra with complete replacement of the osseous matrix in the majority of the vertebral body and posterior elements with a sclerotic inferior and anterior osseous remnant. To assess the histological diagnosis a biopsy was attempted but due to the hard density of the mass was unsuccessful. A marginal tumor resection was performed and stabilization with T1-T6 laminar hook and posterior rods. Pathologic staining and immunoreactivity confirmed a diagnosis of desmoplastic fibroma. Conclusion Desmoplastic fibromas represent an extremely rare primary osseous tumor and localization to the thoracic spine has only been described in a few cases reports. Due to the paucity of cases the radiologic characteristics have not been fully delineated. A combination of CT and MR imaging is recommended in the literature for full characterization of the osseous matrix and evaluation of intramedullary involvement for pre-operative planning. A wide margin of excision is recommended as tumor recurrence has been reported to be between 37-72%. In cases where en bloc resection is not performed, follow up with contrast enhanced MR is recommended for continued surveillance. Now, two years post-resection, the patient has no radiologic evidence of recurrence and is pain-free, working and living an active life. References Bohm P. Desmoplastic fibroma of the bone: A report of two patients, review of the literature and therapeutic implications. Cancer: 78:1011-23, 1996. Crim JR. Desmoplastic fibroma of bone: radiographic analysis. Radiology 172:827-32, 1989. Haney, J. Radiologic case study. The clinical and radiologic features of desmoplastic fibroma of bone. Orthopedics 17:77,80-5,88, 1994. Hardes, J. Three-level En Bloc Spondylectomy for Desmoplastic Fibroma of the Thoracic Spine: A Case Report. Spine 28:E169-72, 2003. Juergens, KU. Desmoplastic fibroma in the thoracic spine: an unusual localization of a rare primary bone tumor. European Radiology 11:2, 273-5, 2001. Krakovits, GE Desmoplastic fibroma in the spine: A case report. Spine 16:481-2, 1991. Schinomiya, K. Desmoplastic fibroma in the thoracic spine. Journal Spinal Disorders 4: 229-33, 1991. Taconis, WK. Desmoplastic fibroma of bone: A report of 18 cases. Skeletal Radiology 23: 282-8, 1994. Vanhoenacker, FM. Desmoplastic fibroma of bone: MRI features. Skeletal Radiology 29:171-5, 2000. Zhang F. Desmoplastic fibroma of the cervical spine case report and review of the literature. Spine. 2010 Jun 15:35 (14) E667-71.