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The clinicoradiological features and surgical outcomes of primary intracranial fibrosarcoma: a single-institute experience with a systematic review

  • Xiu-Jian Ma
  • Da Li
  • Huan Li
  • Liang Wang
  • Shu-Yu Hao
  • Li-Wei Zhang
  • Jun-Ting Zhang
  • Zhen WuEmail author
Original Article
  • 21 Downloads

Abstract

Primary intracranial fibrosarcoma (PIF) was a rare tumor with a high relapse rate and dismal survival rate. This study aimed to delineate the clinical characteristics of primary intracranial fibrosarcoma (PIF) and the risk factors for outcomes. We reviewed 15 PIF patients, who underwent surgical treatment at our institution from January 2009 to December 2018. Meanwhile, 36 cases from the prior literature between November 1962 and December 2019 were also retrieved and pooled to identify the risk factors. In our cohort, while cystic component (46.7%), perilesional edema (83.3%), and vascular flow void (66.7%) were commonly observed, no patient was accurately diagnosed. The 2-year relapse-free survival (RFS) and overall survival (OS) were 12.2% and 30.2%, respectively. Based on the pooled data, tumor size (p = 0.006), Ki-67 index (p = 0.004), and radiotherapy dose (p = 0.029) were prognostic factors for RFS in univariate analysis. In the univariate analysis, tumor size (p = 0.002), NGTR (p = 0.049), and high Ki-67 index (p = 0.019) were significant predictors for OS; and further multivariate analysis (n = 18) showed that large tumor size (≥ 5 cm; HR 14.613, p = 0.022) and high Ki-67 index (≥ 30%; HR 5.879, p = 0.020) were the independent risk factors for OS. Due to the rarity and nonspecific clinicoradiological features, the correct diagnosis of PIF before surgery was challenging. The outcomes of PIF were poor, and GTR plus radiotherapy (at least 60 Gy) might benefit to the outcomes and were recommended. Future study with a large cohort was needed to verify our findings.

Keywords

Outcomes Primary intracranial fibrosarcoma Radiological characteristics Radiotherapy Risk factors Surgery 

Notes

Acknowledgments

We thank senior statistician Dr. Dan Xiao (from the Department of Epidemiology, China National Clinical Research Center for Neurological Disease) and Dr. Wei Feng (from the Department of Epidemiology and Health Statistics, School of Public Health, Capital Medical University) for supervising and improving the confidence of statistical analysis. We also appreciate Pros. Guilin Zhang and Jiang Du (both from Neuropathological Department, Beijing Tiantan Hospital, Capital Medical University) for their great assistance in pathological analyses.

Funding information

This work was supported in part by the National Natural Science Foundation of China awarded to J.T.Z (grant no.81672506) and Z.W. (grant no. 814742370).

Compliance with ethical standards

Conflict of interest

We declare that there is no conflict of interest.

Ethical approval

This study was approved by the Institutional Review Board.

Informed consent

The informed consents were available for all patients.

Supplementary material

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of NeurosurgeryBeijing Tiantan Hospital, Capital Medical UniversityBeijingPeople’s Republic of China
  2. 2.Division of Molecular Neurogenetics, German Cancer Research Center (DKFZ)DKFZ-ZMBH AllianceHeidelbergGermany

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