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

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.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3

References

  1. 1.

    Adeleye AO, Fellig Y, Umansky F, Shoshan Y (2009) Rapid growth of primary cerebral fibrosarcoma with conversion to glioblastoma at second recurrence. J Neurooncol 92:233–238. https://doi.org/10.1007/s11060-008-9756-5

    Article  PubMed  Google Scholar 

  2. 2.

    Aung TH, Tse CH (1993) Bifrontal meningeal fibrosarcoma in a patient with metastases to the liver, kidneys and suprarenal glands. Aust N Z J Surg 63:746–748. https://doi.org/10.1111/j.1445-2197.1993.tb00508.x

    CAS  Article  PubMed  Google Scholar 

  3. 3.

    Bisogno G, Roganovic J, Carli M, Scarzello G, Calderone M, Faggin R, Perilongo G (2002) Primary intracranial fibrosarcoma. Childs Nerv Syst 18:648–651. https://doi.org/10.1007/s00381-002-0628-6

    Article  PubMed  Google Scholar 

  4. 4.

    Brohi SR, Dilber M (2012) Congenital osteolytic dural fibrosarcoma presenting as a scalp swelling. J Coll Physicians Surg Pak 22:531–532. https://doi.org/08.2012/jcpsp.531532

  5. 5.

    Cai N, Kahn LB (2004) A report of primary brain fibrosarcoma with literature review. J Neurooncol 68:161–167. https://doi.org/10.1023/B:NEON.0000027740.70380.5c

    Article  PubMed  Google Scholar 

  6. 6.

    Cassady JR, Wilner HI (1967) The angiographic appearance of intracranial sarcomas. Radiology 88:258–263. https://doi.org/10.1148/88.2.258

    CAS  Article  PubMed  Google Scholar 

  7. 7.

    Chopra R, Bhardwaj M, Premsagar IC (2010) Fibrosarcoma of the meninges. Rare Tumors 2:e3. https://doi.org/10.4081/rt.2010.e3

    Article  PubMed  PubMed Central  Google Scholar 

  8. 8.

    Donnet A, Figarella-Branger D, Grisoli F (1999) Primary meningeal fibrosarcoma: a particular neuroradiological presentation. J Neurooncol 42:79–83. https://doi.org/10.1023/a:1006100119406

    CAS  Article  PubMed  Google Scholar 

  9. 9.

    Fraser RW, Limentani SA, Dollar JD, Asher A (2003) Recurrent primary fibrosarcoma of the brain treated with the GliaSite brachytherapy system: case report. Surg Neurol 60:579–583; discussion 583-574. https://doi.org/10.1016/S0090-3019(03)00456-7

    Article  PubMed  Google Scholar 

  10. 10.

    Gaspar LE, Mackenzie IR, Gilbert JJ, Kaufmann JC, Fisher BF, Macdonald DR, Cairncross JG (1993) Primary cerebral fibrosarcomas. Clinicopathologic study and review of the literature. Cancer 72:3277–3281. https://doi.org/10.1002/1097-0142(19931201)72:11<3277::aid-cncr2820721124>3.0.co;2-c

    CAS  Article  PubMed  Google Scholar 

  11. 11.

    Giridhar P, Mallick S, Haresh KP, Gupta S, Julka PK, Rath GK (2016) Intracranial fibrosarcoma treated with adjuvant radiation and temozolomide: report of a case and review of all published cases. J Egypt Natl Canc Inst 28:111–116. https://doi.org/10.1016/j.jnci.2015.07.002

    Article  PubMed  Google Scholar 

  12. 12.

    Khan WA, Attal H, Vernick J, Bedrossian CW (2001) Cytodiagnosis of a meningeal fibrosarcoma metastatic to the thyroid gland. Semin Diagn Pathol 18:104–109. https://doi.org/10.1053/sdia.2001.23741

    CAS  Article  PubMed  Google Scholar 

  13. 13.

    Kishikawa T, Numaguchi Y, Fukui M, Komaki S, Ikeda J, Kitamura K, Matsuura K (1981) Primary intracranial sarcomas: radiological diagnosis with emphasis on arteriography. Neuroradiology 21:25–31

    CAS  Article  Google Scholar 

  14. 14.

    Lee JG, Song SW, Koh YC, Cho J, Choi JW, Roh HG, Lim SD (2013) Primary intracranial fibrosarcoma presenting with hemorrhage. Brain Tumor Res Treat 1:91–94. https://doi.org/10.14791/btrt.2013.1.2.91

    Article  PubMed  PubMed Central  Google Scholar 

  15. 15.

    Li ZJ, Sun P, Guo Y, Wang RZ (2010) Primary pituitary fibrosarcoma presenting with multiple metastases: a case report and literature review. Neurol India 58:316–318. https://doi.org/10.4103/0028-3886.63792

    Article  PubMed  Google Scholar 

  16. 16.

    Liu Z, Salamat MS, Hafez GR, Mehta MP, Robins HI, Amoli RS, Tomic S (2002) Pineal sarcoma. Acta Neurochir 144:89–92. https://doi.org/10.1007/s007010200008

    CAS  Article  PubMed  Google Scholar 

  17. 17.

    Lopes MB, Lanzino G, Cloft HJ, Winston DC, Vance ML, Laws ER Jr (1998) Primary fibrosarcoma of the sella unrelated to previous radiation therapy. Mod Pathol 11:579–584

    CAS  PubMed  Google Scholar 

  18. 18.

    McDonald P, Guha A, Provias J (1997) Primary intracranial fibrosarcoma with intratumoral hemorrhage: neuropathological diagnosis with review of the literature. J Neurooncol 35:133–139

    CAS  Article  Google Scholar 

  19. 19.

    Onofrio BM, Kernohan JW, Uihlein A (1962) Primary meningeal sarcomatosis. A review of the literature and report of 12 cases. Cancer 15:1197–1208. https://doi.org/10.1002/1097-0142(196211/12)15:6<1197::aid-cncr2820150617>3.0.co;2-1

    CAS  Article  PubMed  Google Scholar 

  20. 20.

    Orbach D, Rey A, Cecchetto G, Oberlin O, Casanova M, Thebaud E, Scopinaro M, Bisogno G, Carli M, Ferrari A (2010) Infantile fibrosarcoma: management based on the European experience. J Clin Oncol 28:318–323. https://doi.org/10.1200/JCO.2009.21.9972

    Article  PubMed  Google Scholar 

  21. 21.

    Paulus W, Slowik F, Jellinger K (1991) Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18:395–402. https://doi.org/10.1111/j.1365-2559.1991.tb00869.x

    CAS  Article  PubMed  Google Scholar 

  22. 22.

    Rovirosa A, Vicente P, Bellmut J, Morales S, Del Campo JM, Bodi R, Ortega A (1992) Meningeal fibrosarcoma: a case report and review of the literature. Acta Neurol Belg 92:30–35

    CAS  PubMed  Google Scholar 

  23. 23.

    Tomita T, Gonzalez-Crussi F (1984) Intracranial primary nonlymphomatous sarcomas in children: experience with eight cases and review of the literature. Neurosurgery 14:529–540. https://doi.org/10.1227/00006123-198405000-00001

    CAS  Article  PubMed  Google Scholar 

  24. 24.

    Torres G, Petit F, Vilchez V, Romero Z, Dorfman S, Cardozo D, Cardozo J (2007) Primary cerebral fibrosarcoma in a child. Clin Neuropathol 26:284–287

    CAS  Article  Google Scholar 

  25. 25.

    Vatsal DK, Sharma S, Renjen PN, Kaul S, Jha AN (2000) Primary fibrosarcoma of brain. Neurol India 48:396–398

    CAS  PubMed  Google Scholar 

  26. 26.

    Vinodh VP, Harun R, Sellamuthu P, Kandasamy R (2017) Primary central nervous system fibrosarcoma. J Neurosci Rural Pract 8:S111–s113. https://doi.org/10.4103/jnrp.jnrp_165_17

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  27. 27.

    Zhang N, Guo L, Kuang H, Ji Y, Zeng X (2019) Primary intracranial fibrosarcoma: case report and systematic review of literature. World Neurosurg 123:251–255. https://doi.org/10.1016/j.wneu.2018.12.005

    Article  PubMed  Google Scholar 

Download references

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

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).

Author information

Affiliations

Authors

Corresponding author

Correspondence to Zhen Wu.

Ethics declarations

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.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

ESM 1

(JPG 1026 kb)

ESM 2

(DOCX 48 kb)

ESM 3

(DOCX 48 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Ma, XJ., Li, D., Li, H. et al. The clinicoradiological features and surgical outcomes of primary intracranial fibrosarcoma: a single-institute experience with a systematic review. Neurosurg Rev 44, 543–553 (2021). https://doi.org/10.1007/s10143-020-01249-8

Download citation

Keywords

  • Outcomes
  • Primary intracranial fibrosarcoma
  • Radiological characteristics
  • Radiotherapy
  • Risk factors
  • Surgery