Primary intradural extramedullary Ewing sarcoma: A case report and literature review

Izubuchi,Yuya; Nakajima,Hideaki; Honjoh,Kazuya; Imamura,Yoshiaki; Nojima,Takayuki; Matsumine,Akihiko

doi:10.3892/ol.2020.11786

Primary intradural extramedullary Ewing sarcoma: A case report and literature review

Authors:
- Yuya Izubuchi
- Hideaki Nakajima
- Kazuya Honjoh
- Yoshiaki Imamura
- Takayuki Nojima
- Akihiko Matsumine
View Affiliations

Affiliations: Department of Orthopaedics and Rehabilitation Medicine, University of Fukui Faculty of Medical Sciences, Eiheiji, Fukui 910‑1193, Japan, Division of Diagnostic Pathology/Surgical Pathology, University of Fukui Hospital, Eiheiji, Fukui 910‑1193, Japan, Department of Pathology, Kanazawa University, Kanazawa, Ishikawa 920‑8641, Japan
Published online on: June 26, 2020 https://doi.org/10.3892/ol.2020.11786
Pages: 2347-2355
Copyright: © Izubuchi et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Tumors of the spinal cord and cauda equina show a wide spectrum of histology and require careful diagnosis and treatment. Primary intradural extramedullary Ewing sarcoma (IEES) is extremely rare, and initial imaging and clinical findings for this tumor mimic those of benign intradural spinal tumors. The present report describes a case of a 35‑year‑old woman who presented with IEES with meningeal seeding, and the literature on the management and clinical course of these tumors was reviewed. An examination revealed right‑side leg dominant rapidly progressive hypoesthesia and motor deficits. MRI identified intradural tumors at the T12‑L1 and L4‑5 levels. Growth and an increase in the number of intradural tumors occurred over 3 weeks. Gadolinium‑contrast T1‑weighted MRI revealed diffusely enhanced signals for tumorous lesions with meningeal seeding in the cervical and thoracic spinal cord. Excision of a tumor at T12‑L1 was performed to alleviate the symptoms and to make a histologic diagnosis. Microscopically, the tumor consisted of dense sheets of small round cells. Immunohistochemically, tumor cells showed intense and diffuse positive staining for CD99, ETS transcription factor ERG and Fli‑1 proto‑oncogene, ETS transcription factor (FLI1). The sequence analysis revealed the EWS RNA binding protein 1‑FLI1 fusion transcript. The lesion was diagnosed as primary intradural ES. Adjuvant chemotherapy following radiotherapy for the whole spine was performed; however, multiple brain metastases were found at 10 months after diagnosis and the patient died of diffusely disseminated disease limited to the central nervous system without evidence of distant metastases at 16 months after the initial diagnosis. In a literature review of IEES cases, the 1‑ and 5‑year overall survival rates were 79.8 and 26.6%, respectively, and the 1‑, 2‑ and 5‑year progression‑free survival rates were 61.0, 52.3 and 10.9%, respectively. Therefore, primary IEES has a poor prognosis compared with ES of bone, and novel agents and treatment strategies are required.

View Figures

View References

1	Van Goethem JW, van den Hauwe L, Ozsarlak O, De Schepper AM and Parizel PM: Spinal tumors. Eur J Radiol. 50:159–176. 2004. View Article : Google Scholar : PubMed/NCBI
2	Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H and Iwamoto Y: Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: Report of four cases and a review of the literature. Spine. 28:E408–E412. 2003. View Article : Google Scholar : PubMed/NCBI
3	Balamuth NJ and Womer RB: Ewings sarcoma. Lancet Oncol. 11:184–192. 2010. View Article : Google Scholar : PubMed/NCBI
4	Gaspar N, Hawkins DS, Dirksen U, Lewis IJ, Ferrari S, Le Deley MC, Kovar H, Grimer R, Whelan J, Claude L, et al: Ewing sarcoma: Current management and future approaches through collaboration. J Clin Oncol. 33:3036–3046. 2015. View Article : Google Scholar : PubMed/NCBI
5	Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC and Dubois SG: Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer. 117:3027–3032. 2011. View Article : Google Scholar : PubMed/NCBI
6	Hisaoka M, Hashimoto H and Murao T: Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Arch. 431:365–369. 1997. View Article : Google Scholar : PubMed/NCBI
7	Isotalo PA, Agbi C, Davidson B, Girard A, Verma S and Robertson SJ: Primary primitive neuroectodermal tumor of the cauda equina. Hum Pathol. 31:999–1001, 200. View Article : Google Scholar : PubMed/NCBI
8	Uesaka T, Amano T, Inamura T, Ikezaki K, Inoha S, Takamatsu M, Iwaki T and Fukui M: Intradural, extramedullary spinal Ewings sarcoma in childhood. J Clin Neurosci. 10:122–125. 2003. View Article : Google Scholar : PubMed/NCBI
9	Akyüz M, Demiral AN, Gürer IE, Uçar T and Tuncer R: Primary primitive neuro-ectodermal tumor of cauda equina with intracranial seeding. Acta Neurochir (Wien). 146:525–528. 2004. View Article : Google Scholar : PubMed/NCBI
10	Mobley BC, Roulston D, Shah GV, Bijwaard KE and McKeever PE: Peripheral primitive neuroectodermal tumor/Ewings sarcoma of the craniospinal vault: Case reports and review. Hum Pathol. 37:845–853. 2006. View Article : Google Scholar : PubMed/NCBI
11	Haresh KP, Chinikkatti SK, Prabhakar R, Rishi A, Rath GK, Sharma DN and Julka PK: A rare case of intradural extramedullary Ewings sarcoma with skip metastasis in the spine. Spinal Cord. 46:582–584. 2008. View Article : Google Scholar : PubMed/NCBI
12	Kim SW and Shin H: Primary intradural extraosseous Ewings sarcoma. J Korean Neurosurg Soc. 45:179–181. 2009. View Article : Google Scholar : PubMed/NCBI
13	Klimo P Jr, Codd PJ, Grier H and Goumnerova LC: Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 4:222–229. 2009. View Article : Google Scholar : PubMed/NCBI
14	Yan Y, Xu T, Chen J, Hu G and Lu Y: Intraspinal Ewings sarcoma/primitive neuroectodermal tumors. J Clin Neurosci. 18:601–606. 2011. View Article : Google Scholar : PubMed/NCBI
15	Vincentelli F, Caruso G and Figarella-Branger D: Primary intradural Ewings sarcoma of the cauda equina presenting with acute bleeding. Acta Neurochir (Wien). 152:563–564. 2010. View Article : Google Scholar : PubMed/NCBI
16	Duan XH, Ban XH, Liu B, Zhong XM, Guo RM, Zhang F, Liang BL and Shen J: Intraspinal primitive neuroectodermal tumor: Imaging findings in six cases. Eur J Radiol. 80:426–431. 2011. View Article : Google Scholar : PubMed/NCBI
17	Karikari IO, Mehta AI, Nimjee S, Hodges TR, Tibaleka J, Montgomery C, Simpson L, Cummings TJ and Bagley CA: Primary intradural extraosseous Ewing sarcoma of the spine: Case report and literature review. Neurosurgery. 69:E995–E999. 2011. View Article : Google Scholar : PubMed/NCBI
18	Pancucci G, Simal-Julian JA, Plaza-Ramirez E, García-Marcos R, Mayordomo-Aranda E and Botella-Asunción C: Primary extraosseous intradural spinal Ewings sarcoma: Report of two cases. Acta Neurochir (Wien). 155:1229–1234. 2013. View Article : Google Scholar : PubMed/NCBI
19	Khalatbari MR, Jalaeikhoo H and Moharamzad Y: Primary intradural extraosseous Ewings sarcoma of the lumbar spine presenting with acute bleeding. Br J Neurosurg. 27:840–841. 2013. View Article : Google Scholar : PubMed/NCBI
20	Bazzocchi A, Bacci A, Serchi E, Salerno A, Salizzoni E and Leonardi M: Intradural extramedullary Ewings sarcoma. Recurrence with acute clinical presentation and literature review. Neuroradiol J. 26:476–481. 2013. View Article : Google Scholar : PubMed/NCBI
21	Gong HS, Huang QS, Liu GJ, Chen FH and Zhao HB: Cervical primary Ewings sarcoma in intradural and extramedullary location and skip metastasis to Cauda Equina. Turk Neurosurg. 25:943–947. 2015.PubMed/NCBI
22	Lozupone E, Martucci M, Rigante L, Gaudino S, Di Lella GM and Colosimo C: Magnetic resonance image findings of primary intradural Ewing sarcoma of the cauda equina: Case report and review of the literature. Spine J. 14:e7–e11. 2014. View Article : Google Scholar : PubMed/NCBI
23	Zhao M, Zhang B, Liang F and Zhang J: Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: Case report and review of the literature. Int J Clin Exp Pathol. 7:9081–9085. 2014.PubMed/NCBI
24	Bostelmann R, Leimert M, Steiger HJ, Gierga K and Petridis AK: The importance of surgery as part of multimodal therapy in rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space. Clin Pract. 6:8972016. View Article : Google Scholar : PubMed/NCBI
25	Kartal A and Akatlı A: Primary intradural extraosseous Ewings sarcoma in a young child. Childs Nerv Syst. 32:409–410. 2016. View Article : Google Scholar : PubMed/NCBI
26	Chihak MA, Ahmed SK, Lachance DH, Nageswara Rao AA and Laack NN: Patterns of failure and optimal radiotherapy target volumes in primary intradural extramedullary Ewing sarcoma. Acta Oncol. 55:1057–1061. 2016. View Article : Google Scholar : PubMed/NCBI
27	Scantland JT, Gondim MJ, Koivuniemi AS, Fulkerson DH and Shih CS: Primary spinal intradural extraosseous Ewing sarcoma in a pediatric patient: Case report and review of the literature. Pediatr Neurosurg. 53:222–228. 2018. View Article : Google Scholar : PubMed/NCBI
28	Paterakis K, Brotis A, Tasiou A, Kotoula V, Kapsalaki E and Vlychou M: Intradural extramedullary Ewings sarcoma: A case report and review of the literature. Neurol Neurochir Pol. 51:106–110. 2017. View Article : Google Scholar : PubMed/NCBI
29	Takami H, Kumar R, Brown DA and Krauss WE: Histologic features and prognosis of spinal intradural extramedullary ewing sarcoma: Case report, literature review, and analysis of prognosis. World Neurosurg. 115:448–452.e2. 2018. View Article : Google Scholar : PubMed/NCBI
30	Tan CH, Tan D, Phung TB and Lai LT: Primary intradural extramedullary Ewing sarcoma of the cervical spine: A case report and review of the literature. J Clin Neurosci. 66:280–284. 2019. View Article : Google Scholar : PubMed/NCBI
31	Fletcher CDM, Bridge JA, Hogendoorn PCW and Mertens F: WHO Classification of Tumours of Soft Tissue and Bone. 4th. IARC; Lyon, France: 2013
32	Zhang J, Huang Y, Sun Y, He A, Zhou Y, Hu H, Yao Y and Shen Z: Impact of chemotherapy cycles and intervals on outcomes of nonspinal Ewing sarcoma in adults: A real-world experience. BMC Cancer. 19:11682019. View Article : Google Scholar : PubMed/NCBI
33	El Weshi A, Allam A, Ajarim D, Al Dayel F, Pant R, Bazarbashi S and Memon M: Extraskeletal Ewings sarcoma family of tumours in adults: Analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol). 22:374–381. 2010. View Article : Google Scholar : PubMed/NCBI