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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 2  |  Page : 257-259

Ewing's sarcoma of the spine: Misdiagnosed as tuberculosis and presenting as paraplegia


1 Department of Medical Oncology, Yenepoya Medical College, Derlakatte, Mangalore, Karnataka, India
2 Department of Orthopaedics, Yenepoya Medical College, Derlakatte, Mangalore, Karnataka, India

Date of Web Publication27-Dec-2018

Correspondence Address:
Dr. Guruprasad Bhat
1-15-c-2, Durga Nivas, Ashraya Nagar, Idya, Kadambodi, Suratkal, Mangalore - 575 014, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/amhs.amhs_1_18

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  Abstract 


Ewing's sarcoma of the spine is a rare tumor, which usually presents with nonspecific symptoms. In India, these are usually misdiagnosed as tuberculosis due to associated fever. These present late with paraplegia. Even then aggressive multimodality therapy of surgery, radiotherapy, and chemotherapy can lead to the recovery of neurological disabilities. This case report highlights the need for prompt diagnosis, multimodality therapy, and complete neurological recovery even when presentation is delayed by 6 months.

Keywords: Recovery, spinal Ewing's sarcoma, tuberculosis


How to cite this article:
Bhat G, Padmanabh S. Ewing's sarcoma of the spine: Misdiagnosed as tuberculosis and presenting as paraplegia. Arch Med Health Sci 2018;6:257-9

How to cite this URL:
Bhat G, Padmanabh S. Ewing's sarcoma of the spine: Misdiagnosed as tuberculosis and presenting as paraplegia. Arch Med Health Sci [serial online] 2018 [cited 2019 Jul 15];6:257-9. Available from: http://www.amhsjournal.org/text.asp?2018/6/2/257/248650




  Introduction Top


Ewing's sarcoma of the spine are rare tumors, which usually presents with non specific symptoms. In India, these are usually misdiagnosed as tuberculosis due to associated fever. These present late with paraplegia. Even when diagnosis is delayed, aggressive multi-modality therapy of surgery, radiotherapy and chemotherapy can lead to recovery of neurological disabilities.


  Case Report Top


a 14-year-old male child presented with 6 month history of fever, back pain and weakness of lower limb to his nearby doctor. He was diagnosed as spinal tuberculosis and was started on antitubercular therapy for 1 month. Since his condition worsened in the form of worsening weakness in lower limb, he was referred to our center for further care. On presentation, he was bed bound, with decreased sensory ability below T9. Magnetic resonance imaging showed an altered signal intensity in the left posterolateral intraspinal location with the extension of lesion into the left neural foramen at T10–T11 vertebra. Altered marrow signal intensity was noted in the left transverse process, bilateral lamina, and spinous process of T10 vertebra [Figure 1]. These were more suggestive of inflammatory/tubercular pathology. Since the patient was already on antitubercular therapy and not improving with therapy, parents were counseled for surgery. The patient underwent surgery as a first modality of management both for tissue diagnosis and stabilization of the spine. He underwent posterior decompression and tumor debulking.
Figure 1: Magnetic resonance imaging showing lesion of T10 vertebra

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The postoperative histology was suggestive of small round cell neoplasm. Moreover, the final histopathology was suggestive of Ewing's sarcoma (ES). Immunohistochemistry was done, it was CD99 strong positive further confirming the diagnosis. Metastatic workup included a computed tomography scan of the chest, bone marrow examination, and bone scan; all of which were normal. He was started on alternate cycles of vincristine, cyclophosphamide and adriamycin till 375 mg/m2 then actinomycin D/alternating with ifosfamide and etoposide given every 3 weeks for a total of seventeen cycles as per first Pediatric Oncology Group–Children's Cancer Group protocol. After nine cycles of chemotherapy, he underwent radiotherapy to the spine (40 Gy/25#/over 5 weeks).

The patient became ambulatory within 4 months of treatment initiation for ES [Figure 2]. He has completed treatment and presently under follow-up since the last 9 months.
Figure 2: The patient able to walk without support

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  Discussion Top


ES was first described by James Ewing in 1921. This small round-cell tumor family consists of ES, primitive neuroectodermal tumor, and Askins tumor. This is the second most common pediatric bone tumor, and it predominantly involves the extremities, pelvis, or thorax. It rarely involves the flat bones and vertebra. Spinal ES presents in thoracic vertebra as epidural lesion or paraspinal soft-tissue masses with secondary spinal involvement. These rarely present in other location such as cervical, lumbar, intradural, or intramedullary lesions. Spinal Ewing's sarcoma was first described in 1945, in a 21-year-old male patient.[1]

Fever is the initial presentation in 30%–50% of cases of Ewings sarcoma.[2] Since the symptoms are nonspecific, they are misdiagnosed in up to 80% of times in the first visit.[3] They are usually misdiagnosed as Tuberculosis in India, as it is endemic here.[4]

When the patient is neurologically stable, usually a guided large bore needle biopsy will help in diagnosis. Moreover, these patients are usually started on neoadjuvant chemotherapy followed by definitive local therapy in the form of both radiotherapy and surgery. In these patients, local therapy in the form of radiotherapy and surgery is usually done between 9 and 12 weeks. Radiotherapy can be given alone as a form of local therapy; however, these patients may have a higher recurrence rate if residual tumor >10%.

When the patient is neurologically unstable usually surgery is the preferred upfront modality. As these tumors presents as paravertebral soft-tissue masses which invade the spinal canal through the intervertebral foramina and compress the spine circumferentially, laminectomy is an effective approach for stabalization.[5] Chemotherapy regimen is similar to ES of other sites. Neoadjuvant chemotherapy can be given for bulky tumors. Our case report highlights the need for correct diagnosis. The presence of fever may lead to false diagnosis of infectious etiology. Early initiation of multimodality therapy can lead to better oncological control and neurological recovery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patients has/have given their consent for 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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Imler AE, Meilstrup DB, Bogart FB. Primary Ewing's sarcoma of the spine. Radiology 1946;46:597-600.  Back to cited text no. 1
    
2.
Grier HE. The Ewing family of tumors. Ewing's sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 1997;44:991-1004.  Back to cited text no. 2
    
3.
Widhe B, Widhe T, Bauer HC. Ewing sarcoma of the rib – Initial symptoms and clinical features: Tumor missed at the first visit in 21 of 26 patients. Acta Orthop 2007;78:840-4.  Back to cited text no. 3
    
4.
Rajappa S, Menon PG, Sundaram S. Ewings sarcoma of the hand-a case report. J Hand Microsurg 2010;2:82-4.  Back to cited text no. 4
    
5.
Dini LI, Mendonça R, Gallo P. Primary Ewings sarcoma of the spine: Case report. Arq Neuropsiquiatr 2006;64:654-9.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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