Surgical Management of Craniospinal Axis Solitary Fibrous Tumors: A Single-Institution Case Series and Comprehensive Review of the Literature

Abstract

Background and objectives: Meningeal solitary fibrous tumors (SFTs) comprise 0.4% of primary central nervous system neoplasms and carry metastatic potential. Disease course and optimal management are largely unknown, and there is currently no literature rigorously describing neurological outcomes in surgically managed SFTs. We present one of the largest craniospinal SFT series, analyze patient outcomes, and extensively review the associated literature.

Methods: All surgically managed SFTs at our institution between January 2005 and March 2023 were retrospectively reviewed. Patient demographics, tumor and radiographic features, treatment, and clinical outcomes were collected. Neurological function was quantified using Frankel grade and Neurologic Assessment in Neuro-Oncology scores. Descriptive statistics, multivariate analysis, log-rank test, and Kaplan-Meier survival analysis were performed.

Results: Twenty-one patients satisfied inclusion criteria. Tumor locations included 15 supratentorial, three infratentorial, and three spinal. All patients underwent surgical resection, and 16 (76.2%) underwent radiation. Six (28.6%) patients had tumor recurrence, and three (14.3%) developed metastasis. Younger age and higher postoperative Frankel grade were significantly associated with increased overall survival (OS) ( P = .011, P = .002, respectively). All patients symptomatically improved or stabilized after surgery, and Neurologic Assessment in Neuro-Oncology score ( P = .001) and functional status significantly improved postoperatively (Karnofsky Performance Status: 65.2 ± 25.2 vs 91.4 ± 13.5, P = .001). Sex, adjuvant radiation, and extent of resection were not significantly associated with OS.

Conclusion: SFT of the central nervous system is a rare entity with a variable clinical course. Surgical resection was associated with improved postoperative functional and neurological status. Higher postoperative neurological function was significantly associated with OS. Further studies are warranted to validate a standardized treatment algorithm and investigate the efficacy of adjuvant radiation in SFT.

FIGURE 1.

Kaplan–Meier survival curves displaying association A, between age (P = .01), B, postoperative Frankel grade (P = .002), C, EOR (P = .19), D, sex (P = .26), E, adjuvant radiation (P = .16), and F, tumor grade (P = .58) and OS. EOR, extent of resection; OS, overall survival.

FIGURE 2.

A, Gross appearance of spinal SFT after dural opening and exposure. B, Spinal SFT, after resection, is solid and relatively firm with minimal prominence of vascularity. C and D, Sagittal pre- and postoperative T1-weighted MRI with gadolinium contrast images demonstrating a round T9-T10 hyperintense lesion in the spinal canal, respectively. Hematoxylin and eosin, E, 100× magnification: SFT with typical haphazardly arranged cells within a background of thin-walled, dilated vessels and F, 200× magnification: area of tumor necrosis supportive of CNS WHO grade III designation. Cd, caudal; Cr, cranial; D, dura mater; Sc, spinal cord; SFT, solitary fibrous tumor.

FIGURE 3.

MRI of brain showing well-circumscribed extra-axial mass in the right frontal lobe with avid heterogeneous enhancement in A, axial T-1 postgadolinium and B, axial T2 sequences. Prominent flow voids are also seen in these areas (blue arrows). MRI of postgadolinium contrast T1 sequence of the spine showing an intradural extramedullary mass at T5-6 on C, axial and D, sagittal views of a different patient.