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Review
. 2022 Jun 8;14(12):2839.
doi: 10.3390/cancers14122839.

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Caroline Apra  1   2 , Amira El Arbi  3 , Anne-Sophie Montero  1   2 , Fabrice Parker  3   4 , Steven Knafo  3   4
Affiliations
Review

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Caroline Apra et al. Cancers (Basel). .

Abstract

All solitary fibrous tumors (SFT), now histologically diagnosed by a positive nuclear STAT6 immunostaining, represent less than 2% of soft tissue sarcomas, with spinal SFT constituting a maximum of 2% of them, making these tumors extremely rare. We provide an up-to-date overview of their diagnosis, treatment, and prognosis. We included 10 primary STAT6-positive SFT from our retrospective cohort and 31 from a systematic review. Spinal pain was the most common symptom, in 69% of patients, and the only one in 34%, followed by spinal cord compression in 41%, radicular compression, including pain or deficit, in 36%, and urinary dysfunction specifically in 18%. Preoperative diagnosis was never obtained. Gross total resection was achieved in 71%, in the absence of spinal cord invasion or excessive bleeding. Histologically, they were 35% grade I, 25% grade II, and 40% grade III. Recurrence was observed in 43% after a mean 5.8 years (1 to 25). No significant risk factor was identified, but adjuvant radiotherapy improved the recurrence-free survival after subtotal resection. In conclusion, spinal SFT must be treated by neurosurgeons as part of a multidisciplinary team. Owing to their close relationship with the spinal cord, radiotherapy should be considered when gross total resection cannot be achieved, to lower the risk of recurrence.

Keywords: STAT6; hemangiopericytoma; intramedullary; medulla; neurosurgery; solitary fibrous tumor; spine.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Recurrence-free survival (Kaplan–Meier curve) for all patients with a spinal solitary fibrous tumor and at least 12 months of follow-up, based on our series and literature systematic review (n = 35). Each + accounts for a patient death or end of follow-up. Left: for patients with gross total resection compared to subtotal resection. Right: for patients with adjuvant radiotherapy compared to no radiotherapy. Radiotherapy significantly improved recurrence-free survival in patients with subtotal resection.
Figure 2
Figure 2
Graphical summary of the characteristics and clinico-radiological presentation of patients with spinal solitary fibrous tumors, including radiological types of SFT on MRI.
Figure 3
Figure 3
Graphical summary of the surgery, diagnosis, adjuvant treatments, and outcome of patients with spinal solitary fibrous tumors.
See this image and copyright information in PMC

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