J Neurol Surg A Cent Eur Neurosurg 2014; 75(06): 485-490
DOI: 10.1055/s-0034-1372437
Case Report
Georg Thieme Verlag KG Stuttgart · New York

A Nonradiated Grade II Glioma That Underwent Delayed Malignant Transformation to a Gliosarcoma with Meningeal Growth and Dissemination

Fabien Rech
1   Department of Neurosurgery, CHU Nancy, Nancy, France
Valerie Rigau
2   Department of Neuropathology, CHU de Montpellier, Montpellier, France
Michel Fabbro
3   Department of Neurooncology, ICM, Montpellier, France
Christine Kerr
3   Department of Neurooncology, ICM, Montpellier, France
Guillaume Gauchotte
4   Department of Neuropathology, CHU Nancy, Nancy, France
Luc Taillandier
5   Department of Neurooncology, CHU Poitiers, Poitiers, France
Hugues Duffau
6   Department of Neurosurgery, Montpellier University Medical Center, Montpellier, France
› Author Affiliations
Further Information

Publication History

17 November 2013

03 January 2014

Publication Date:
27 June 2014 (online)


Background Secondary gliosarcomas are rare tumors, especially those arising from a World Health Organization (WHO) grade II glioma not irradiated. We report a case with subtotal resection for a WHO grade II oligoastrocytoma, without adjuvant treatment, whose metaplastic transformation into gliosarcoma suddenly occurred 4 years later with meningeal dissemination. We show a favorable outcome after therapeutic management of this rare entity.

Patient A 46 year-old woman underwent surgery for a right premotor WHO grade II oligoastrocytoma discovered incidentally. Because of a subtotal resection with only 1 cc of residue, no complementary therapy was given, and the patient enjoyed a normal life for 4 years. In the meantime, the magnetic resonance images performed every 6 months showed a very low growth rate. Suddenly, the tumor switched toward a gliosarcoma profile with meningeal dissemination.

Results Reoperation, radiotherapy, and chemotherapy were performed, enabling a control of the disease with 15 months of follow-up (i.e., with radiologic shrinkage of the multiple lesions and preservation of quality of life).

Conclusion A delayed sarcomatous transformation can acutely occur with a low proliferation index in a nonirradiated WHO grade II oligoastrocytoma. Furthermore, an aggressive therapeutic strategy can allow control of secondary gliosarcomas, even in cases of leptomeningeal spreading.

  • References

  • 1 Louis DN. WHO Classification of Tumours of the Central Nervous System. Lyon, France: International Agency for Research on Cancer; 2007
  • 2 Stroebe H. Uber entstehung und bau der hirngliome. Beitr Pathol Anat 1895; 18: 405-486
  • 3 Galanis E, Buckner JC, Dinapoli RP , et al. Clinical outcome of gliosarcoma compared with glioblastoma multiforme: North Central Cancer Treatment Group results. J Neurosurg 1998; 89 (3) 425-430
  • 4 Meis JM, Martz KL, Nelson JS. Mixed glioblastoma multiforme and sarcoma. A clinicopathologic study of 26 radiation therapy oncology group cases. Cancer 1991; 67 (9) 2342-2349
  • 5 Pallud J, Taillandier L, Capelle L , et al. Quantitative morphological magnetic resonance imaging follow-up of low-grade glioma: a plea for systematic measurement of growth rates. Neurosurgery 2012; 71 (3) 729-739 ; discussion 739–740
  • 6 Han SJ, Yang I, Ahn BJ , et al. Clinical characteristics and outcomes for a modern series of primary gliosarcoma patients. Cancer 2010; 116 (5) 1358-1366
  • 7 Han SJ, Yang I, Tihan T, Prados MD, Parsa AT. Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity. J Neurooncol 2010; 96 (3) 313-320
  • 8 Han SJ, Yang I, Otero JJ , et al. Secondary gliosarcoma after diagnosis of glioblastoma: clinical experience with 30 consecutive patients. J Neurosurg 2010; 112 (5) 990-996
  • 9 Han SJ, Yang I, Tihan T, Chang SM, Parsa AT. Secondary gliosarcoma: a review of clinical features and pathological diagnosis. J Neurosurg 2010; 112 (1) 26-32
  • 10 Rodriguez FJ, Scheithauer BW, Jenkins R , et al. Gliosarcoma arising in oligodendroglial tumors (“oligosarcoma”): a clinicopathologic study. Am J Surg Pathol 2007; 31 (3) 351-362
  • 11 Codispoti KE, Mosier S, Ramsey R, Lin MT, Rodriguez FJ. Genetic and pathologic evolution of early secondary gliosarcoma. Brain Tumor Pathol 2014; 31 (1) 40-46
  • 12 Kobayashi T, Sakai K, Tada T , et al. Gliosarcoma arising from a fibrillary astrocytoma. J Clin Neurosci 2011; 18 (9) 1251-1254
  • 13 Salvati M, Caroli E, Raco A, Giangaspero F, Delfini R, Ferrante L. Gliosarcomas: analysis of 11 cases do two subtypes exist?. J Neurooncol 2005; 74 (1) 59-63
  • 14 Feigin IH, Gross SW. Sarcoma arising in glioblastoma of the brain. Am J Pathol 1955; 31 (4) 633-653
  • 15 Actor B, Cobbers JMJL, Büschges R , et al. Comprehensive analysis of genomic alterations in gliosarcoma and its two tissue components. Genes Chromosomes Cancer 2002; 34 (4) 416-427
  • 16 Biernat W, Aguzzi A, Sure U, Grant JW, Kleihues P, Hegi ME. Identical mutations of the p53 tumor suppressor gene in the gliomatous and the sarcomatous components of gliosarcomas suggest a common origin from glial cells. J Neuropathol Exp Neurol 1995; 54 (5) 651-656
  • 17 Boerman RH, Anderl K, Herath J , et al. The glial and mesenchymal elements of gliosarcomas share similar genetic alterations. J Neuropathol Exp Neurol 1996; 55 (9) 973-981
  • 18 Hiniker A, Hagenkord JM, Powers MP, Aghi MK, Prados MD, Perry A. Gliosarcoma arising from an oligodendroglioma (oligosarcoma). Clin Neuropathol 2013; 32 (3) 165-170
  • 19 Jones H, Steart PV, Weller RO. Spindle-cell glioblastoma or gliosarcoma?. Neuropathol Appl Neurobiol 1991; 17 (3) 177-187
  • 20 Reis RM, Könü-Lebleblicioglu D, Lopes JM, Kleihues P, Ohgaki H. Genetic profile of gliosarcomas. Am J Pathol 2000; 156 (2) 425-432
  • 21 Vajtai I, Vassella E, Hewer E, Kappeler A, Reinert MM. Sarcomatous evolution of oligodendroglioma (“oligosarcoma”): confirmatory report of an uncommon pattern of malignant progression in oligodendroglial tumors. Pathol Res Pract 2012; 208 (12) 750-755
  • 22 Pakos EE, Goussia AC, Zina VP, Pitouli EJ, Tsekeris PG. Multi-focal gliosarcoma: a case report and review of the literature. J Neurooncol 2005; 74 (3) 301-304
  • 23 Demirci S, Akalin T, Islekel S, Ertan Y, Anacak Y. Multiple spinal metastases of cranial gliosarcoma: a case report and review of the literature. J Neurooncol 2008; 88 (2) 199-204
  • 24 Witwer BP, Salamat MS, Resnick DK. Gliosarcoma metastatic to the cervical spinal cord: case report and review of the literature. Surg Neurol 2000; 54 (5) 373-378 ; discussion 378–379
  • 25 Fischer S, Lee W, Aulisi E, Singh H. Gliosarcoma with intramedullary spinal metastases: a case report and review of the literature. J Clin Oncol 2007; 25 (4) 447-449