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.

Intracranial Solitary Fibrous Tumor: A Case of a 21-Year-Old Male With Olfactory Hallucination

“…Among 19 cases of SFTs in the middle cranial fossa reviewed by Maiuri et al, meningioma was the only preoperative diagnosis reported (15 cases were “not specified," and four admitted to suspecting meningioma) [ 4 ]. Another recently published review of 21 craniospinal SFT cases performed by Piscopo et al reported that 10 of their cases received an initial diagnosis of meningioma on radiological examination [ 6 ]. An interesting radiologic difference described in that case series is that the lesions were generally described as heterogenous, whereas the lesion in our patient was more homogenous in appearance.…”

“…An interesting radiologic difference described in that case series is that the lesions were generally described as heterogenous, whereas the lesion in our patient was more homogenous in appearance. Diagnosing SFT appropriately is paramount because, although these tumors are localized at presentation, they carry a significant risk of recurrence and metastasis [ 6 ]. Therefore, maximal safe gross total resection is the goal of treatment with the use of adjuvant radiation for high-grade lesions to minimize this risk [ 6 ].…”

“…Diagnosing SFT appropriately is paramount because, although these tumors are localized at presentation, they carry a significant risk of recurrence and metastasis [ 6 ]. Therefore, maximal safe gross total resection is the goal of treatment with the use of adjuvant radiation for high-grade lesions to minimize this risk [ 6 ].…”

“…The population-based study by Kinslow et al, which serves as one of the few sources of epidemiologic data regarding CNS SFTs, calculated a median age of 54 [3]. Four different case series and single-institution retrospective analyses reported mean ages of 55.4, 46.4, 43, and again 43, with an additional case series reporting a median age of 45.5 [6,[9][10][11][12].…”