Primary Intracranial Sarcomas, Myxoid Meningeal Sarcoma – a Case Report and Literature Review

Download PDF Česká verzia

Authors: D. Vrána ¹; O. Kalita ²; L. Hrabálek ²; M. Matzenauer ¹; M. Bartoušková ¹; N. Rušarová ¹; L. Tučková ³; L. Sedláčková ⁴
Authors place of work: Onkologická klinika LF UP a FN Olomouc ¹; Neurochirurgická klinika LF UP a FN Olomouc ²; Ústav klinické a molekulární patologie, LF UP a FN Olomouc ³; Radiologická klinika LF UP a FN Olomouc ⁴
Published in the journal: Klin Onkol 2019; 32(3): 214-219
Category: Case Report
doi: https://doi.org/10.14735/amko2019214

Summary

Background: Primary intracranial sarcoma is a rare disease. Due to the scarcity of evidence from randomized clinical trials, we follow the treatment guidelines of their extracranial counterparts or those published in case reports, while taking into consideration the specificity of radiotherapy within the brain, and the limit imposed on chemotherapy by the blood brain barrier. Nevertheless, surgery remains the golden standard of treatment for primary tumours, and also for recurrence. Even though there are usually narrow margins achieved in brain compared with the extracranial sarcomas. Despite significant effort, prognosis remains dismal.

Case: We present a 69-year old woman who was investigated for psychoorganic syndrome and paresis of the left hand. Magnetic resonance imaging revealed a tumour expansion in her frontal lobe with collateral oedema. Surgical resection was indicated. Histology of the specimen suggested a myxoid meningeal sarcoma. Early disease recurrence 4 months after primary resection was treated by reresection and 50 Gy of adjuvant radiotherapy to the tumour bed. Similarly, another recurrence 19 months after the second surgery was treated using the same approach. Systemic treatment has not been indicated so far. At this time, the patient is without evidence of any disease recurrence and continues with regular follow-up.

Conclusion: Myxoid meningeal sarcoma represents a rare disease with a high risk of recurrence. Unfortunately, there is no clear recommendation for treatment algorithm.

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.

Keywords:

radiotherapy – brain tumors – sarcomas

Zdroje

1. Maher OM, Khatua S, Mukherjee D et al. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature. J Neurooncol 2016; 127 (1): 155–163. doi: 10.1007/s11060-015-2027-3.

2. Louis DN, Perry A, Reifenberger G et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 2016; 131 (6): 803–820. doi: 10.1007/s00401-016-1545-1.

3. Benesch M, von Bueren AO, Dantonello T et al. Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups. J Neurooncol 2013; 111 (3): 337–345. doi: 10.1007/s11060-012-1020-3.

4. UpToDate.com. Uncommon brain tumors. [online]. Available from: https: //www.uptodate.com/contents/uncommon-brain-tumors?search=brain%20tumors&topicRef=5180&source=see_link#H45.

5. Davis JL, Tihan, T, Kilpatrick, SE. Mesenchymal tumors of the central nervous system. In: Perry A, Brat DJ (eds). Practical surgical neuropathology: a diagnostic approach. 2nd ed. Radarweg: Elsevier 2018: 299–322.

6. Kalita O, Cwiertka K, Vrána D et al. Multimodal therapy of recurrent malignant schwannoma. Klin Onkol 2016; 29 (5): 364–368. doi: 10.14735/amko2016364.

7. Kawabata Y, Aoki T, Yamamoto T et al. Pazopanib-mediated long-term disease stabilization after local recurrence and distant metastasis of primary intracranial leiomyosarcoma: a case report on the efficacy of pazopanib as a salvage therapy. NMC Case Rep J 2017; 5 (1): 1–7. doi: 10.2176/nmccrj.cr.2017-0048.

8. Apra C, Alentorn A, Mokhtari K et al. Pazopanib efficacy in recurrent central nervous system hemangiopericytomas. J Neurooncol 2018; 139 (2): 369–372. doi: 10.1007/s11060-018-2870-0.

9. Lafay-Cousin L, Lindzon G, Taylor MD et al. Successful treatment of primary intracranial sarcoma with the ICE chemotherapy regimen and focal radiation in children. J Neurosurg Pediatr 2016; 17 (3): 298–302. doi: 10.3171/2015.6.PEDS14709.

10. Merimsky O, Lepechoux C, Terrier P, et al. Primary sarcomas of the central nervous system. Oncology 2000; 58 (3): 210–214. doi: 10.1159/000012102.

11. Patel M, Li L, Nguyen HS et al. Primary intracranial synovial sarcoma. Case Rep Neurol Med 2016; 2016: 5608315. doi: 10.1155/2016/5608315.

12. Wushou A, Zhao YJ, Shao ZM. Synovial sarcoma of the infratemporal fossa: a case report. Oncol Lett 2014; 8 (5): 2165–2170. doi: 10.3892/ol.2014.2436.

13. Garciaz S, Bouvier C, Roche PH et al. Primary dediffentiated liposarcoma of the dura mater: case report. Acta Neurochir (Wien) 2012; 154 (9): 1737–1738. doi: 10.1007/s00701-012-1449-3.

14. Mumert ML, Walsh MT, Jensen EM et al. Pleomorphic liposarcoma originating from intracranial dura mater. J Neurooncol 2010; 97 (1): 149–153. doi: 10.1007/s11060-009-0005-3.

15. Hussain S, Nanda A, Fowler M et al. Primary intracranial leiomyosarcoma: report of a case and review of the literature. Sarcoma 2006; 2006: 52140. doi: 10.1155/SRCM/2006/52140.

16. Lee TT, Page LK. Primary cerebral leiomyosarcoma. Clin Neurol Neurosurg 1997; 99 (3): 210–212.

17. Torihashi K, Chin M, Yoshida K et al. Primary intracranial leiomyosarcoma with intratumoral hemorrhage: case report and review of literature. World Neurosurg 2018; 116: 169–173. doi: 10.1016/j.wneu.2018.05.004.

18. Gallagher SJ, Rosenberg SA, Francis D et al. Primary intracranial leiomyosarcoma in an immunocompetent patient: case report and review of the literature. Clin Neurol Neurosurg 2018; 165: 76–80. doi: 10.1016/j.clineuro.2017.12.014.

19. Kumar V, Singh A, Sharma V et al. Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: a rare tumor with review of literature. Asian J Neurosurg 2017; 12 (3): 351–357. doi: 10.4103/1793-5482.185060.

20. Choudhury KB, Sharma S, Kothari R et al. Primary extraosseous intracranial Ewing’s sarcoma: case report and literature review. Indian J Med Paediatr Oncol 2011; 32 (2): 118–121. doi: 10.4103/0971-5851.89798.

21. Dedeurwaerdere F, Giannini C, Sciot R et al. Primary peripheral PNET/Ewing’s sarcoma of the dura: a clinicopathologic entity distinct from central PNET. Mod Pathol 2002; 15 (6): 673–678. doi: 10.1038/modpathol.3880 585.

22. Palta M, Riedel RF, Vredenburgh JJ et al. Primary meningeal rhabdomyosarcoma. Sarcoma 2011; 2011: 312802. doi: 10.1155/2011/312802.

23. Zhang S, Ju Y, You C. A rare case of extensive primary meningeal osteosarcoma in childhood. Neurology 2016; 87 (13): 1420–1421. doi: 10.1212/WNL.0000000000003159.

24. Romeo E, Gisserot O, de Jaureguiberry JP et al. Meningeal chondroblastic osteosarcoma: case report and review of the literature. J Neurooncol 2010; 100 (2): 305–309. doi: 10.1007/s11060-010-0167-z.

25. Dagcinar A, Bayrakli F, Yapicier O et al. Primary meningeal osteosarcoma of the brain during childhood. Case report. J Neurosurg Pediatr 2008; 1 (4): 325–329. doi: 10.3171/PED/2008/1/4/325.

26. Setzer M, Lang J, Turowski B et al. Primary meningeal osteosarcoma: case report and review of the literature. Neurosurgery 2002; 51 (2): 488–492, discussion 492.

27. Sadashiva N, Sharma A, Shukla D et al. Intracranial extraskeletal mesenchymal chondrosarcoma. World Neurosurg 2016; 95: 618.e1–618.e6. doi: 10.1016/j.wneu.2016.08.049.

28. Kotil K, Bilge T, Olagac V. Primary intradural myxoid chondrosarcoma: a case report and review in the lite-rature. J Neurooncol 2005; 75 (2): 169–172. doi: 10.1007/ s11060-005-1739-1.

29. Qin Y, Zhang HB, Ke CS et al. Primary extraskeletal myxoid chondrosarcoma in cerebellum: a case report with literature review. Medicine (Baltimore) 2017; 96 (47): e8684. doi: 10.1097/MD.0000000000008684.

30. Li D, Weng JC, Zhang GJ et al. Proposed treatment paradigm for intracranial chondrosarcomas based on multidisciplinary coordination. World Neurosurg 2018; 109: e517–e530. doi: 10.1016/j.wneu.2017.10.013.

31. Beatty Z, Bergman T. Meningeal hemangiopericytoma with intracranial metastases in an HIV-positive male: case report and review of the literature. Case Rep Oncol 2012; 5 (1): 159–163. doi: 10.1159/000337580.

32. Rutkowski MJ, Sughrue ME, Kane AJ et al. Predictors of mortality following treatment of intracranial hemangiopericytoma. J Neurosurg 2010; 113 (2): 333–339. doi: 10.3171/2010.3.JNS091882.

33. Majumdar K, Mandal S, Saran RK et al. Recurrent intracranial myxofibrosarcoma presenting as an extensive fronto-parieto-occipital SOL: an unusual sarcoma of meningeal origin. Clin Neurol Neurosurg 2013; 115 (3): 354–358. doi: 10.1016/j.clineuro.2012.05.030.

34. Gaspar LE, Mackenzie IR, Gilbert JJ et al. Primary cerebral fibrosarcomas. Clinicopathologic study and review of the literature. Cancer 1993; 72 (11): 3277–3281.

35. Karthigeyan M, Malik P, Garg S et al. Intracranial low grade fibromyxoid sarcoma with an atypical radiology. World Neurosurg 2019; 122: 500–504. doi: 10.1016/j.wneu.2018.11.082.

36. Tun K, Ozen O, Kaptanoglu E et al. Primary intracranial low-grade fibromyxoid sarcoma (Evans tumor). J Clin Neurosci 2008; 15 (11): 1298–1301. doi: 10.1016/j.jocn.2007.07.085.