Desmoplastic fibroma of the spine: a series of 12 cases and outcomes

Abstract

Background context: Desmoplastic fibroma (DF) is a benign, yet locally aggressive, tumor of the connective tissue. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Although surgical resection of DF arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results.

Purpose: This study reviews the clinical patterns and follow-up data of patients with DF in the spine who underwent surgical treatment. We attempted to correlate surgical treatment and outcomes over time.

Study design: A retrospective clinical study of the surgical managements, including subtotal resection, total spondylectomy, and en bloc resection, for DF in the spine. Desmoplastic fibroma of the spine treatment occurred from 2004 to 2009 at the Department of Bone Tumor Surgery, AA Hospital.

Patient sample: Twelve consecutive cases of DF of the spine underwent surgical treatment at our center between 2004 and 2009.

Outcome measures: Neurologic outcomes were evaluated using Frankel score system and recurrence and metastasis were evaluated by computed tomography or magnetic resonance imaging of the surgical segments involved. Imaging was performed 3, 6, and 12 months after surgery, every 6 months for the next 2 years, and then annually for life.

Methods: Overall, two different surgery protocols were applied. One protocol involved subtotal resection followed by radiotherapy (n=4), whereas the other involved total tumor resection (n=8). Postoperative radiotherapy was administered in six cases. Clinical data and surgery efficacy were analyzed via chart review.

Results: Eleven patients were disease-free during their follow-up period, whereas one patient experienced recurrence without metastasis. Radicular pain nearly disappeared, and patients suffering from spinal cord compression recovered well. Local recurrence was detected in one-fourth (25%) of the cases that underwent subtotal resection and was not detected in any of the cases involving total spondylectomy.

Conclusions: Local recurrence of DF is not uncommon after insufficient removal. Therefore, total excision, while also preserving neural function, is recommended. In our study, patients who underwent a total spondylectomy had significantly lower local recurrence rates for DF in the spine. Radiotherapy may be an acceptable alternative therapy, whereas en bloc resection has the potential to result in significant functional impairment.

Keywords: Case series; Desmoplastic fibroma; En bloc resection; Outcomes; Radiotherapy; Spine; Total spondylectomy.