Study design: A case of desmoplastic fibroma of the thoracic spine treated by a three-level en bloc spondylectomy is reported.
Objectives: To present a rare case of desmoplastic fibroma of the spine, and to emphasize the importance of at least a marginal resection of this tumor entity.
Summary of background data: Desmoplastic fibroma is a rare tumor, with the literature reporting approximately 220 cases of the disorder in patients younger than 30 years of age. This disorder has a predilection for the mandible and the meta-diaphyses of long bones. A location of desmoplastic fibroma in the spine has been reported in only a few cases. Desmoplastic fibroma has a high tendency of local recurrence, especially after intralesional resection.
Methods: The reported case is that of a 14-year-old girl with a desmoplastic fibroma of the 9th, 10th, and 11th vertebrae. After confirmation of the diagnosis by CT-guided biopsy, a three-level en bloc spondylectomy with marginal resection of the desmoplastic fibroma was performed from the posterior approach. Stabilization was achieved with a multilevel pedicle screw instrumentation, and an autologous fibula was used for reconstruction.
Results: At this writing, 31 months after surgery, the girl has no evidence of recurrence and is pain free.
Conclusions: Wide resection of tumors located in the spine actually is impossible to achieve because of the anatomic relations to the spinal cord, the major vessels, and the lung. As in the current case, a marginal resection is the maximal one to be achieved. Three of seven cases (43%) of desmoplastic fibroma in the spine treated by intralesional resection resulted in a local recurrence. These data clarify the importance of at least marginal resection of desmoplastic fibroma, if this is anatomically and technically possible. A local recurrence of desmoplastic fibroma in the spine can be impossible to treat surgically with a curative intention without a significant loss of function.