Clinical outcomes in central nervous system solitary-fibrous tumor/hemangiopericytoma: a STROBE-compliant single-center analysis

Abstract

Background: Solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) are rare mesenchymal tumors in the central nervous system with a high tendency to relapse, having a significant impact on quality of life (QoL). Due to the rarity of intracranial SFT/HPC, the prognostic factors and optimal treatment remain to be elucidated. Meanwhile, quality of life in patients with intracranial SFT/HPC is seldomly concerned. Thus, we aim to survey about the quality of life and underline some aspects demanding concern in intracranial SFT/HPC treatment through summarizing our case series in recent ten years.

Methods: Patients with intracranial SFT/HPC who underwent surgical resection from January 2009 to June 2019 were included in the study. Clinical features, such as age, gender, and resection extent, were collected. The EuroQol Five Dimensions Questionnaire (EQ-5D) was used to assess the patients' quality of life (QoL). Prognosis factors related to progression-free survival (PFS) and overall survival (OS) were also evaluated.

Results: Thirty-six patients with a mean follow-up period of 61.6 months (range 13-123 months) were included in this study. Sixteen (44.4%) patients achieved gross total resection (GTR). Fourteen patients (38.9%) with tumor progression experienced adjuvant radiotherapy (11.1%) or Gamma Knife surgery (GKS, 27.8%). According to the 2016 WHO classification, there were 6 (16.7%) grade I SFT/HPC, 11 (30.5%) grade II SFT/HPC, and 19 (52.8%) grade III SFT/HPC. The PFS and OS were 29 months (range 4-96 months) and 38 months (range 4-125 months). The median EQ5D-3 L tariff with or without progression was 0.617 (95% CI 0.470-0.756) and 0.939 (95% CI 0.772-0.977) respectively. Gross total resection (GTR, p = 0.024) and grade I SFT/HPC (p = 0.017) were significantly associated with longer PFS. In multivariate analysis, GTR (HR 0.378, 95% CI 0.154-0.927) and adjuvant therapy (HR 0.336, 95% CI 0.118-0.956) result in significantly longer PFS in patients with SFT/HPC.

Conclusions: Patients underwent GTR and adjuvant therapy had longer PFS. Similarly, patients with lower WHO grade had relatively longer PFS. Therefore, GTR is advocated for the treatment of SFT/HPC. And adjuvant therapy such as GKS could be an alternative treatment for patients who underwent STR or with tumor progression. Further, the QoL decreased in patients with tumor progression and metastasis, and more attention is demanded to the QoL of intracranial SFT/HPC patients.

Keywords: Central nervous system; Hemangiopericytoma; Prognosis; Quality of life; Solitary fibrous tumor.

Fig. 1

The flowchart of enrollment and follow-up. Initially, 39 patients were identified according to postoperative pathology results of SFT/HPC. Then, two patients were excluded for incomplete information and one for the extracranial lesion. Three patients lost response before the last follow-up for QoL. Moreover, eight patients had been dead before the last follow-up. Two patients did not respond to the investigation of QoL at the last follow-up

Fig. 2

Representative CT and MRI images before and after operation. a Preoperative CT and vascular reconstruction images exhibited the SFT/HPC lesion in the left middle cranial fossa. This irregular high-density mass showing heterogeneous enhancement had unclear boundaries and destroyed the surrounding skull. And this lesion partially enveloped the left internal carotid artery segments C3-C4. b The preoperative enhanced T1WI images were displayed. The mass was located in the left middle cranial fossa, with unclear boundaries and heterogeneous enhancement. The lesion protruded locally into the left pontine corner cistern, partially compressed the left temporal lobe and brainstem and enveloped the C3-C4 segments of the left internal carotid artery. c The enhanced T1WI images of 1-year past operation were exhibited. The area of the original lesion showed postoperative changes without significant enhancement

Fig. 3

Kaplan-Meier survival curves display over all-survival and progression-free survival with different EOR and WHO grade. Figures exhibited EOR (a, b) and WHO grade (c, d)-related overall-survival and progression-free survival. EOR and WHO grade in PFS were statistically significant with p-value equaling 0.024 and 0.017. GTR, gross total resection; STR, subtotal resection