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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1405-1408

Primary Spinal Intradural Ewing's Sarcoma: Hemorrhagic Presentation with Acute Neurological Deterioration in Two Pediatric Patients

1 Department of Neurosurgery, PGIMER, Chandigarh, India
2 Department of Neurosurgery, KGMU, Lucknow, Uttar Pradesh, India
3 Department of Pathology, KGMU, Lucknow, Uttar Pradesh, India
4 Department of Pathology, PGIMER, Chandigarh, India

Date of Submission08-May-2019
Date of Decision18-Jul-2019
Date of Acceptance28-Jun-2021
Date of Web Publication30-Oct-2021

Correspondence Address:
Sushanta K Sahoo
Department of Neurosurgery, PGIMER, Sector 12, Chandigarh - 160 0l2
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.329577

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 » Abstract 

Extraosseous primary spinal intradural Ewing's sarcoma (ES) is an unusual entity. Bleed within such tumors with acute neurological worsening is extremely rare, especially in the pediatric age. In this article, we present two children with intradural-extramedullary ES who had sudden decline in their neurological status consequent to an intratumoral hemorrhage. We discuss their clinical course and also briefly review the pertinent literature. Spinal intradural Ewing's sarcomas possibly have a tendency to bleed. A short clinical symptomatology along with an acute neurological deterioration and radiologic evidence of intratumoral hemorrhage in a spinal intradural tumor should raise the suspicion of an ES.

Keywords: Ewing's sarcoma, intra dural extra medullary, spinal cord tumor
Key Message: Spinal intra dural Ewings sarcoma is unusual. In pediatric patients they may present with acute deterioration due to intra tumoral bleed. They have dismal prognosis even after early intervention.

How to cite this article:
Karthigeyan M, Malik P, Sahoo SK, Salunke P, Vankipuram S, Sagar M, Das A. Primary Spinal Intradural Ewing's Sarcoma: Hemorrhagic Presentation with Acute Neurological Deterioration in Two Pediatric Patients. Neurol India 2021;69:1405-8

How to cite this URL:
Karthigeyan M, Malik P, Sahoo SK, Salunke P, Vankipuram S, Sagar M, Das A. Primary Spinal Intradural Ewing's Sarcoma: Hemorrhagic Presentation with Acute Neurological Deterioration in Two Pediatric Patients. Neurol India [serial online] 2021 [cited 2022 May 25];69:1405-8. Available from: https://www.neurologyindia.com/text.asp?2021/69/5/1405/329577

Ewing's sarcoma (ES), which belongs to the category of small round cell neoplasms frequently affects children aged less than 20 years and usually involves the long bones and pelvic region.[1],[2],[3] Its extraosseous form is relatively rare and accounts for approximately 20% of cases.[4] In spine, it often presents as paravertebral and epidural lesions.[5],[6],[7],[8],[9] Infrequently, ES can present as intradural tumors. An associated intratumoral bleed is extremely rare.[10],[11],[12] The literature on such manifestations is sparse, particularly in children.[11] Herein, we report a couple of intradural extramedullary pediatric ES that had acute neurological deterioration secondary to bleed within and discuss their clinical course and the relevant literature.

 » Case Report Top

Patient 1

A 5-year-old girl presented with insidious-onset paraparesis of 1-month duration. On examination, she had mild limping during walking. Magnetic resonance imaging (MRI) revealed a large intradural-extramedullary tumor extending from D10-L3 levels causing expansion of spinal cord and compression of cauda equina. The lesion was of intermediate signal on T1, hyperintense on T2/STIR images, and showed intense contrast enhancement [Figure 1]. A myxopapillary ependymoma was kept as a differential.
Figure 1: (a and b) (Case 1), MRI showing intradural tumor extending from D10 to L3 vertebral levels causing expansion of spinal cord and compression of cauda equine nerve roots. The tumor was hyperintense on T2 weighted sequence (a), and showed intense contrast enhancement with few small areas of non-enhancement (b). (c) (Case 2) Contrast enhancing cervicodorsal intradural lesion from C6-D3 levels

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While the child was under evaluation, there was sudden worsening of weakness to flaccid paraplegia along with urinary retention over a span of few hours. She underwent urgent D10-L3 laminoplasty. After durotomy, the tumor started bulging out with evidence of multiple areas of hemorrhage [Figure 2]. It was mildly vascular, soft-suckable, and interspersed between the nerve roots. The caudal end of the spinal cord and conus region appeared edematous even after complete tumor excision. A lax duraplasty was performed.
Figure 2: (a and b) Intraoperative photographs (a, case 1; b, case 2) showing hemorrhagic lesion with clots protruding out of dura, with evidence of bleed

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Histopathology revealed tumor cells arranged in form of diffuse sheets, clusters, and rosette formation with large areas of necrosis. They stained positive for CD99 and negative for CD45, FLI1, desmin, and myogenin. The Ki67 index was 20% [Figure 3]. The features were suggestive of ES. A systemic work-up excluded disseminated disease. The child received adjuvant chemoradiation. However, she showed no improvement at 6-month follow-up.
Figure 3: (a) Histopathological section at low power view shows small round cells densely packed in sheets, and around the blood vessels (red arrow) along with necrotic areas (black arrow). H and E100X. (b) Section shows rosettes formation (arrow) by small round cells. H and E200X. (c) High power view shows round to oval cells forming rosettes, and with conspicuous nucleoli and scant amount of cytoplasm. H and E400X. (d and e) Immunohistochemistry was strongly positive for Vimentin (d) and CD99 (e). (f) Section shows nuclear positivity for Ki67

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Patient 2

A 3-year-old boy presented to the emergency department with complaints of breathlessness for 2 days. There was progressive weakness of all limbs over the past 2 weeks. He had spastic quadriparesis with power 3/5. MRI showed a contrast-enhancing intradural cervico-dorsal lesion extending from C6-D3 level, suggestive of a meningioma [Figure 1]. During surgery, we noticed a thick clot protruding out with evidence of intratumoral bleed [Figure 2]. The tumor was firm, vascular, and suckable and complete excision could be achieved. Postoperatively, the child required mechanical ventilation and expired on postoperative day 14. Histopathological analysis revealed ES [Figure 3].

 » Discussion Top

ES is a highly malignant primary bone tumor, the origin of which is controversial.[13] Both neuroectodermal and mesenchymal cell lineages have been proposed. At times, ES may origin from paravertebral region, soft tissues of lower limbs, head and neck, chest wall, and retroperitoneum.[8]

Spine is a less frequent site and involved only in 3.5%–7% of patients. Frequently, these are epidural lesions or an extension from the vertebral body.[7],[9] Primary spinal intradural ES is uncommon and less than 30 cases have been reported.[9],[11] Though common in lumbar area, it can involve any part of spinal axis. ES usually affects children and young adults with a median age of 32 years.

In sporadic reports, primary spinal intradural ES have been shown acute neurological manifestations like cauda equine syndrome.[9] Such case in pediatric population is rare and only one case with acute paraparesis due to L2-3 lesion has been described in past.[11] These patients presented with cauda equina syndrome, acute paraplegia, or severe low backache. Our cases also suggest that these intradural tumors have a propensity to bleed. This possibly attributed to the highly proliferative nature of the tumor causing hemorrhagic necrosis.[6]

On imaging, they show iso-hyperintense on T1 and hyperintense on T2 sequences. With contrast, they display homogenous or heterogenous enhancement; a heterogenous enhancement might suggest hemorrhage or necrosis.[14] It is difficult to differentiate spinal intradural ES from ependymomas. The latter is also commonly seen in children and shares similar imaging features. Occasionally, spinal ependymomas are also known to present with hemorrhagic manifestations.[15] ES can also resemble other common intradural tumors like schwannomas and meningiomas as seen in our patient. A hemorrhage within the spinal intradural tumor might hint toward an ES although it is rare. ES should be considered as a differential, especially when accompanied with short clinical symptomatology and sudden neurological worsening.

ES is an aggressive tumor and requires multimodal approach with surgery, chemotherapy, and radiotherapy. With the advent of newer chemotherapy, extraskeletal ES has shown a more favorable outcome as compared with skeletal ES.[4],[16] In localized disease, such as the spinal ES, additional investigations are required to exclude systemic/metastatic disease. The management of spinal ES is maximal safe resection along with adjuvant chemoradiation.[6] In primary intradural ES, the 5-year overall survival rate was lower (43%) compared with that of the localized extraskeletal and skeletal ES (69.7% and 62.6%, respectively).[7],[16] The clinical experience for tumors that present with acute neurological worsening is currently limited owing to the rarity of such cases. Although the children described here showed poor neurological outcome, few reports have documented an improvement in motor power and bladder function after surgery.[6],[9] A possible reasoning for the observed difference could be the severity of the deficits at presentation. Before the surgical decompression, the child (Patient 1) had already suffered a complete spinal deficit as compared with those with less severe neurological grades reported from other studies. A timely intervention could possibly alter the postoperative neurology in such patients.

 » Conclusions Top

Although rare, extraosseous ES can present as primary spinal intradural lesions and resemble the commonly occurring intradural-extramedullary neoplasms. An evidence of hemorrhage on imaging and/or during surgery might suggest an ES in pediatric age group. Considering the fact, that these tumors tend to bleed and present with acute neurological deterioration, an early surgery is preferred. A high clinico-radiological suspicion could possibly alter the postoperative neurological outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, et al. Prognostic factors in Ewing's tumor of bone: Analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. J Clin Oncol 2000;18:3108-14.  Back to cited text no. 1
Ozaki T. Diagnosis and treatment of Ewing sarcoma of the bone: A review article. J Orthop Sci 2015;20:250-63.  Back to cited text no. 2
Salunke PS, Gupta K, Malik V, Kumar N, Henke LE, Cai C, et al. Primary Ewing's sarcoma of cranial bones: Analysis of ten patients. Acta Neurochir (Wien) 2011;153:1477-85.  Back to cited text no. 3
Cash T, McIlvaine E, Krailo MD, Lessnick SL, Lawlor ER, Laack N, et al. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group. Pediatr Blood Cancer 2016;63:1771-9.  Back to cited text no. 4
Kim SW, Shin H. Primary intradural extraosseous Ewing's sarcoma. J Korean Neurosurg Soc 2009;45:179-81.  Back to cited text no. 5
Pancucci G, Simal-Julian JA, Plaza-Ramirez E, García-Marcos R, Mayordomo-Aranda E, Botella-Asunción C. Primary extraosseous intradural spinal Ewing's sarcoma: Report of two cases. Acta Neurochir (Wien) 2013;155:1229-34.  Back to cited text no. 6
Takami H, Kumar R, Brown DA, Krauss WE. Histologic features and prognosis of spinal intradural extramedullary Ewing sarcoma: Case report, literature review, and analysis of prognosis. World Neurosurg 2018;115:448-52.  Back to cited text no. 7
Bazzocchi A, Bacci A, Serchi E, Salerno A, Salizzoni E, Leonardi M. Intradural extramedullary Ewing's sarcoma. Recurrence with acute clinical presentation and literature review. Neuroradiol J 2013;26:476-81.  Back to cited text no. 8
Krishnakumar R, Renjitkumar J. Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine. Neurol India 2011;59:931-3.  Back to cited text no. 9
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Khalatbari MR, Jalaeikhoo H, Moharamzad Y. Primary intradural extraosseous Ewing's sarcoma of the lumbar spine presenting with acute bleeding. Br J Neurosurg 2013;227:840-1.  Back to cited text no. 10
Scantland JT, Gondim MJ, Koivuniemi AS, Fulkerson DH, Shih CS. Primary spinal intradural extraosseous Ewing sarcoma in a pediatric patient: Case report and review of the literature. Pediatr Neurosurg 2018;53:222-8.  Back to cited text no. 11
Vincentelli F, Caruso G, Figarella-Branger D. Primary intradural Ewing's sarcoma of the cauda equina presenting with acute bleeding. Acta Neurochir (Wien) 2010;152:563-4.  Back to cited text no. 12
Lin PP, Wang Y, Lozano G. Mesenchymal stem cells and the origin of Ewing's sarcoma. Sarcoma 2011;2011. doi: 10.1155/2011/276463.  Back to cited text no. 13
Javery O, Krajewski K, O'Regan K, Kis B, Giardino A, Jagannathan J, et al. A to Z of extraskeletal Ewing sarcoma family of tumors in adults: Imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol 2011;197:1015-22.  Back to cited text no. 14
Tait MJ, Chelvarajah R, Garvan N, Bavetta S. Spontaneous hemorrhage of a spinal ependymoma: A rare cause of acute cauda equina syndrome: A case report. Spine (Phila Pa 1976) 2004;29:E502-5.  Back to cited text no. 15
Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011;117:3027-32.  Back to cited text no. 16


  [Figure 1], [Figure 2], [Figure 3]


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