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LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 1  |  Page : 249-251

Sacral Ewing's tumor: Use of neoadjuvant chemotherapy for preoperative cytoreduction of the tumor


Department of Neurosurgery, Sakra World Hospital, Devarabeesanahalli, Bengaluru, Karnataka, India

Date of Web Publication11-Jan-2018

Correspondence Address:
Dr. Venkata Ramakrishna Tukkapuram
Department of Neurosurgery, Sakra World Hospital, Devarabeesanahalli, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.222848

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How to cite this article:
Tukkapuram VR, Rudrappa S, Shabadi VK, Masapu D, Kumar S. Sacral Ewing's tumor: Use of neoadjuvant chemotherapy for preoperative cytoreduction of the tumor. Neurol India 2018;66:249-51

How to cite this URL:
Tukkapuram VR, Rudrappa S, Shabadi VK, Masapu D, Kumar S. Sacral Ewing's tumor: Use of neoadjuvant chemotherapy for preoperative cytoreduction of the tumor. Neurol India [serial online] 2018 [cited 2019 Jul 17];66:249-51. Available from: http://www.neurologyindia.com/text.asp?2018/66/1/249/222848




Sir,

A 14-year old boy presented with pain in the sacral region for 22 days, which was radiating to the left thigh, along with a history of intermittent high-grade fever that reduced with medication. He first identified the pain while playing. On examination, the patient was conscious and oriented; bilateral lower limb numbness was present, left > right, and, his straight leg raising test was negative; his upper limb and lower limb reflexes were normal. The Babinski's sign was equivocal and he had no bowel and bladder incontinence. The patient was investigated with plain radiographs, a computed tomography (CT) scan, and a magnetic resonance imaging (MRI), which showed the lesion in the sacrum with a wide extension. The MRI showed the destructive sacral mass involving the mid and left half of the sacral ala, extending from S2 to S5 levels, with involvement of all sacral nerve roots except the S1 nerve root [Figure 1].
Figure 1: a. CT scan shows the lesion in the sacrum with a wide extension. b. MRI shows the destructive sacral mass involving the mid and left half of the sacral ala from S2 to S5 level with involvement of all sacral nerve roots except S1

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Considering the age of the patient, his subtle clinical symptoms as well as his radiological findings, the differential diagnoses included chordoma, tuberculosis, lymphoma, and Ewing's sarcoma. Metastasis was not considered as a differential diagnosis due to his young age. The need for further investigations, such as a positron emission tomography (PET) CT, was discussed with the tumor board and was not done preoperatively as the patient was young, and metastasis was not considered in the differential diagnosis. However, metastatic foci of Ewing's tumor are not uncommon.[1],[2] A biopsy was performed, and the histopathological findings were suggestive of a round cell tumor. Involvement of additional bones by the Ewing's tumor was ruled out clinically and radiologically utilizing plain radiographs and the CT scan images.

The tumor had a wide extension involving bilateral S2 nerve roots. Considering the poor prognosis, especially in sacral Ewing's tumor, and due to the chemosensitive nature of the round cell tumor, the patient was advised neoadjuvant chemotherapy for cytoreduction. The standard vincristine, adriamycin and cyclophosphamide (VAC) regimen has been considered as being effective for an Ewing's tumor at different locations. Recent studies, however, have shown that administering ifosfamide and itoposide demonstrates better results compared to the VAC [3] regimen. The patient was given 6 cycles of ifosfamide and itoposide over a period of 12 weeks preoperatively, as advised by the tumor board.[4]

Post-chemotherapy radiographs and the CT scan showed a significant lesion reduction. In consonance with this finding, a repeat MRI scan showed 80% tumor shrinkage [Figure 2].
Figure 2: a. Postchemotherapy CT scan showed the lesion reduction, b. MRI pelvis showed the lesion involving the left half of the sacral ala extending from the S2 to S5 vertebrae, with 80% of tumour shrinkage after chemotherapy

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The patient was then posted for partial sacrectomy.[5] Under general anesthesia, with the patient being in a prone position, partial sacrectomy was done under neuromonitoring guidance, which included anal sphincter function monitoring and neuronavigation. The anesthesia technique used was total intravenous anesthesia with no usage of a relaxant during the procedure.

During surgery, the sacrum was exposed using a midline exposure. The identification of the S2 nerve roots and preserving them were the crucial steps in the preservation of bowel and bladder functions. The S2 nerve roots were identified by gently lifting the pyriformis muscle, separating it from the nerve roots on either side, and then incising it. The level of the sacrum to be resected was identified by navigation. As the tumor was extending until the left S2 nerve, the nerve roots were stimulated with a bipolar probe at 5 mA current and the evoked potentials elicited using the electromyographic procedure were noted [Figure 3], [Figure 4], [Figure 5]. The left S2 nerve root was sacrificed as we could not elicit any evoked potentials from it. However, the right S2 nerve root was carefully preserved. The resected sacrum was gently lifted off from the tumor bed after releasing the adhesions and then removed without injuring the rectum. The soft tissue extension of the tumor around the sacrum was then resected and the wound closed in layers. The patient was mobilized on the first postoperative day. He had no bladder incontinence and there were no fresh deficits. Eight more cycles of chemotherapy were advised in the postoperative period to address the residual tumor and to prevent a tumor relapse and metastasis.
Figure 3: Exposure and identifying S2 nerve root by lifting the pyriformis muscle. The nerve roots are stimulated with a bipolar probe starting with 5 milliamperes current, and noting down evoked potentials generated on the electromyogram

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Figure 4: Sacrum after resection

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Figure 5: Resecting the soft tissue extension of the tumour

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In our report, a 14-year old boy presented with a 3-week history of pain radiating to the left thigh. MRI was suggestive of a sacral chordoma. Due to the extensive nature of the tumor, the differential diagnoses including infective [6],[7] causes, osteosarcoma, lymphoma,[7] and Ewing's tumor [8],[9]. Although MRI was sensitive in detecting the lesion at an early stage, it was not specific and confirmative; hence, establishment of a histopathological diagnosis was mandatory to provide an optimal and appropriate treatment.

Ewing's sarcoma is a malignant round cell neoplasm of the bone. The spinal column is involved by a primary Ewing's sarcoma in 10% of the total cases with this pathology.[10] Its occurrence in the sacrum is very rare. Sacral tumors mostly present with subtle symptoms initially, such as localized mild pain in the sacral region, but once the tumor aggressively infiltrates the surrounding regions, radicular symptoms with neurological deficits set in. Bowel and bladder disturbances may also occur mainly caused by the involvement of S2 and S3 nerve roots. Radiologically, it presents as a lytic, sclerotic, or a mixed lesion extending to the surrounding soft tissue structures.[11],[12],[13],[14]

Ewing's sarcoma is chemosensitive (with the incidence of local recurrence being 6.25% and only 25% of patients suffering from this entity having a long-term survival),[15] and therefore, chemotherapy should be an integral part of management of these lesions. as surgery alone does not give satisfactory results. Therefore, once the diagnosis of sacral Ewing's sarcoma was established, the sacrectomy was postponed and neoadjuvant chemotherapy was given.

In conclusion, preoperative neoadjuvant chemotherapy helps in minimizing the complications of surgery by shrinking the chemosensitive tumor, thereby reducing the extent of resection required in patients suffering from a sacral Ewing's sarcoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Bacci G, Boriani S, Balladelli A, Barbieri E, Longhi A, Alberghini M, et al. Treatment of nonmetastatic Ewing's sarcoma family tumors of the spine and sacrum: The experience from a single institution. Eur Spine J 2009;18:1091-5.  Back to cited text no. 2
    
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Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003;348:694-701.  Back to cited text no. 3
    
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Diel J, Ortiz O, Losada RA, Price DB, Hayt MW, Katz DS. The sacrum: Pathologic spectrum, multimodality imaging, and subspecialty approach 1. Radiographics 2001;21:83-104.  Back to cited text no. 8
    
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Jain AK. Tuberculosis of the spine. J Bone Joint Surg Br 2010;92:905-13.  Back to cited text no. 9
    
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Srinivasalu S, D'Souza A. Sacral Ewing's sarcoma and challenges in its diagnosis on MRI. J Radiol Case Rep 2009;3:23-6.  Back to cited text no. 10
    
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Nair P, Aggarwal V, Kumar K, Mathews A, Abraham M, Nair S. Calvarial Ewing's sarcoma, a rare childhood tumor: Report and a review of literature. Neurol India 2017;65:904-6.  Back to cited text no. 13
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Amita R, Sandhyamani S, Nair S, Kapilamoorthy TR, Amita R, Sandhyamani S, Nair S, Kapilamoorthy T R. Intracranial Ewings sarcoma/peripheral primitive neuroectodermal tumor. Neurol India 2014;62:432-3.  Back to cited text no. 14
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Gopalakrishnan CV, Shrivastava A, Easwer HV, Nair S. Primary Ewing's sarcoma of the spine presenting as acute paraplegia. J Pediatr Neurosci 2012;7:64-6.  Back to cited text no. 15
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