A Patient with Primary Intraventricular Gliosarcoma and Long-term Survival – a Case Report

Česká verzia

Authors: O. Kalita ¹; M. Zlevorová ²; M. Megová ³; M. Vaverka ¹; R. Trojanec ³; L. Tučková ⁴
Authors place of work: Neurochirurgická klinika LF UP a FN Olomouc ¹; Onkologická klinika LF UP a FN Olomouc ²; Laboratoř experimentální medicíny, Ústav molekulární a translační medicíny, LF UP a FN Olomouc ³; Laboratoř molekulární patologie, Oddělení patologie, LF UP a FN Olomouc ⁴
Published in the journal: Klin Onkol 2016; 29(6): 454-459
Category: Kazuistika
doi: https://doi.org/10.14735/amko2016454

Summary

Background:
Gliosarcoma is a rare, malignant CNS tumor with a very poor prognosis. Gliosarcoma is a variant of glioblastoma multiforme, which is characterized by the presence of both glial and mesenchymal components. The treatment strategy for gliosarcomas has not yet been determined clearly.

Case presentation:
This case report presents a 23-year-old female patient who complained of increasing headaches, nausea and vomiting, and slight motor weakness in her left arm. An MRI scan of the brain showed a tumor filling the anterior part of the right lateral ventricle and extending into the right frontal lobe. Tumor extirpation was performed. Histology revealed gliosarcoma. Subsequently, the patient received concomitant chemoradiotherapy with temozolomide in the Stupp regimen. Following the fourth cycle of maintenance temozolomide chemotherapy, at eight months after diagnosis, an MRI scan detected progression of the tumor residue. The patient underwent another surgery and then received 10 cycles of second-line chemotherapy in the ICE (ifosfamide, carboplatin, and etoposide) regimen. She completed oncological therapy with minimal toxicity and follow-up MRI scans showed virtually no residual tumor. Another follow-up MRI scan, performed 28 months after diagnosis, demonstrated progression of the tumor residue again. A third tumor resection was performed 29 months after initial diagnosis. Histology again confirmed gliosarcoma. An early postoperative MRI scan showed subtotal resection with a tumor residue in eloquent areas and also suspected implantation metastasis in the spinal canal at the C2 level. From the neurological perspective, the patient was fully self-sufficient, and had only a very mild motor deficit in her left arm. Currently, at 31 months after initial diagnosis, the patient is in a stable condition and fully self-sufficient.

Conclusion:
Our case report shows that long-term survival can be achieved in a gliosarcoma patient exhibiting all the unfavorable features in clinical-pathological terms. The minimal recommended treatment is maximal resection followed by adjuvant radiotherapy. Our patient also underwent chemoradiotherapy with temozolomide in the Stupp regimen. Recurrence at eight months after diagnosis was managed by a repeat operation and high-dose combination chemotherapy, which kept the disease in remission for 20 months after the initial relapse. The lack of unequivocal rules for chemotherapy provides an opportunity to test less common treatment regimens.

Key words:
gliosarcoma – surgery – chemotherapy – radiotherapy – survival

This study was supported in part by the grant No. NT13581-4/2012(86-91) of the Internal Grant Agency of the Czech Ministry of Health.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Submitted:
26. 3. 2016

Accepted:
27. 4. 2016

Zdroje

1. Louis DN, Ohgaki H, Wiestler OD et al. Gliosarcoma. In: WHO classification of tumours of the central nervous system. 4th ed. Lyon: IARC 2007: 48–49.

2. Lutterbach J, Guttenberger R, Pagenstecher A. Gliosarcoma: a clinical study. Radiother Oncol 2001; 61 (1): 57–64.

3. Zhang BY, Chen H, Geng DY et al. Computed tomography and magnetic resonance features of gliosarcoma: a study of 54 cases. J Comput Assist Tomogr 2011; 35 (6): 667–673. doi: 10.1097/RCT.0b013e3182331128.

4. Kozak KR, Mahadevan A, Moody JS. Adult gliosarcoma: epidemiology, natural history, and factors associated with outcome. Neuro Oncol 2009; 11 (2): 183–191. doi: 10.1215/15228517-2008-076.

5. Lee D, Kang SY, Suh YL et al. Clinicopathologic and genomic features of gliosarcomas. J Neurooncol 2012; 107 (3): 643–650. doi: 10.1007/s11060-011-0790-3.

6. Romero-Rojas AE, Diaz-Perez JA, Ariza-Serrano LM et al. Primary gliosarcoma of the brain: radiologic and histopathologic features. Neuroradiol J 2013; 26 (6): 639–648.

7. Han SJ, Yang I, Tihan T et al Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity. J Neurooncol 2010; 96 (3): 313–320. doi: 10.1007/s11060-009-9973-6.

8. Švajdler M, Rychlý B, Gajdoš M et al. Gliosarkóm s komponentou pripomínajúcou alveolárny rabdomyosarkóm: popis prípadu s doposial nepopísanou sarkómovou zložkou. Cesk Patol 2012; 48 (4): 210–214.

9. Reis RM, Könü-Lebleblicioglu D, Lopes JM et al. Genetic profile of gliosarcomas. Am J Pathol 2000; 156 (2): 425–432.

10. Bouchalova K, Trojanec R, Kolar Z et al. Analysis of ERBB2 and TOP2A gene status using fluorescence in situ hybridization versus immunohistochemistry in localized breast cancer. Neoplasma 2006; 53 (5): 393–401.

11. Weisenberger DJ, Campan M, Long TI et al. Analysis of repetitive element DNA methylation by MethyLight. Nucleic Acids Research 2005; 33 (21): 6823–6826.

12. Kristensen LS, Andersen GB, Hager H et al. Competitive amplification of differentially melting amplicons (CADMA) enables sensitive and direct detection of all mutation types by high-resolution melting analysis. Hum Mutat 2012; 33 (1): 264–271. doi: 10.1002/humu.21598.

13. Biswas A, Kumar N, Kumar P et al. Primary gliosarcoma – clinical experience from a regional cancer centre in north India. Br J Neurosurg 2011; 25 (6): 723–729. doi: 10.3109/02688697.2011.570881.

14. Damodaran O, van Heerden J, Nowak AK et al. Clinical management and survival outcomes of gliosarcomas in the era of multimodality therapy. J Clin Neurosci 2014; 21 (3): 478–481. doi: 10.1016/j.jocn.2013.07.042.

15. Winkler PA, Büttner A, Tomezzoli A et al. Histologically repeatedly confirmed gliosarcoma with long survival: review of the literature and report of a case. Acta Neurochir (Wien) 2000; 142 (1): 91–95.

16. Huo Z, Yang D, Shen J et al. Primary gliosarcoma with long-survival: report of two cases and review of literature. Int J Clin Exp Pathol 2014; 7 (9): 6323–6333.

17. Baldawa S, Kasegaonkar P, Vani S et al Primary intraventricular gliosarcoma. Clin Neuropathol 2013; 32 (6): 525–528. doi: 10.5414/NP300607.

18. Moiyadi A, Sridhar E, Jalali R. Intraventricular gliosarcoma: unusual location of an uncommon tumor. J Neurooncol 2010; 96 (2): 291–294. doi: 10.1007/s11060-009-9952-y.

19. Singh G, Das KK, Sharma P et al. Cerebral gliosarcoma: analysis of 16 patients and review of literature. Asian J Neurosurg 2015; 10 (3): 195–202. doi: 10.4103/1793-5482.161173.

20. Salvati M, Caroli E, Raco A et al. Gliosarcomas: analysis of 11 cases do two subtypes exist? J Neurooncol 2005; 74 (1): 59–63.

21. Singh G, Mallick S, Sharma V et al. A study of clinico-pathological parameters and O6-methylguanine DNA methyltransferase (MGMT) promoter methylation status in the prognostication of gliosarcoma. Neuropathology 2012; 32 (5): 534–542. doi: 10.1111/j.1440- 1789.2012.01297.x.

22. Kang SH, Park KJ, Kim CY et al. O6-methylguanine DNA methyltransferase status determined by promoter methylation and immunohistochemistry in gliosarcoma and their clinical implications. J Neuroonco 2011; 101 (3): 477–486. doi: 10.1007/s11060-010-0267-9.

23. Lee D, Kang SY, Suh YL et al. Clinicopathologic and genomic features of gliosarcomas. J Neurooncol 2012; 107 (3): 643–650. doi: 10.1007/s11060-011- 0790-3.

24. Sanai N, Berger MS. Glioma extent of resection and its impact on patient outcome. Neurosurgery 2008; 62 (4): 753–764. doi: 10.1227/01.neu.0000318159.21731.cf.

25. Stupp R, Mason WP, van den Bent MJ et al. Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 2005; 352 (10): 987–996.

26. Perry JR, Ang LC, Bilbao JM et al. Clinicopathologic features of primary and postirradiation cerebral gliosarcoma. Cancer 1995; 75 (12): 2910–2918.

27. Morantz RA, Feigen I, Ransohoff J. Clinical and pathological study of 24 cases of gliosarcoma. J Neurosurg 1976; 45 (4): 398–408.

28. 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. doi: 10.1002/cncr.24857.

29. Walker GV, Gilbert MR, Prabhu SS et al. Temozolomide use in adult patients with gliosarcoma: an evolving clinical practice. J Neurooncol 2013; 112 (1): 83–89. doi: 10.1007/s11060-012-1029-7.

30. Forshew T, Lewis P, Waldman A et al. Three different brain tumours evolving from a common origin. Oncogenesis 2013; 2: e41. doi: 10.1038/oncsis.2013.1.

31. Stančoková T. Liečba dětských nádorov mozgu. Onkologia (Bratisl.) 2007; 2 (3): 176–180.

32. Pavelka Z, Zitterbart K. Nádory centrálního nervového systému u dětí. Neurol pro praxi 2011; 12 (1): 52–58.