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Table of Contents    
LETTER TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 6  |  Page : 1543-1544

A Rare Case of Intradural Extramedullary Ewing's Sarcoma in the Cervical Region


1 Department of Neurosurgery, P D Hinduja Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, P D Hinduja Hospital, Mumbai, Maharashtra, India
3 Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication20-Dec-2019

Correspondence Address:
Dr. Ketan Desai
C/o Marylee, 1st Floor Faculty, Wing Number 4, Hinduja Clinic, Hinduja Hospital, Veer Sawarkar Marg, Mahim, Mumbai - 400 016, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.273622

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How to cite this article:
Warade AC, Jha AK, Pattankar S, Madiwale C, Rekhi B, Desai K. A Rare Case of Intradural Extramedullary Ewing's Sarcoma in the Cervical Region. Neurol India 2019;67:1543-4

How to cite this URL:
Warade AC, Jha AK, Pattankar S, Madiwale C, Rekhi B, Desai K. A Rare Case of Intradural Extramedullary Ewing's Sarcoma in the Cervical Region. Neurol India [serial online] 2019 [cited 2020 Jan 21];67:1543-4. Available from: http://www.neurologyindia.com/text.asp?2019/67/6/1543/273622




Sir,

Ewing's sarcoma was classically described by James Ewing in 1921 as a malignant, osteolytic, round cell tumor of the bone.[1] It commonly affects the long bones and pelvis. Ewing's sarcoma involving the spinal column is not very common and its reported incidence is 3.5–7% in the world literature.[1] The cases have been mostly extradural involving the spinal bones.[1] The occurrence of the intradural, extraosseous Ewing's sarcoma is extremely rare and we report a unique case of intradural extramedullary (IDEM) cervical region Ewing's sarcoma. Till date, only four cases of IDEM Ewing's sarcoma in the cervical region are documented in world literature [2],[3],[4],[5] and we hereby report the fifth case.

A 33-year-old female presented with relatively rapidly worsening neck pain and paraesthesia in the right upper limb for the last one month. On examination, she had a sensory level at C5. There was no motor weakness in the limbs. The deep tendon reflexes were brisk in both upper and lower limbs with extensor plantar reflex bilaterally. Magnetic resonance imaging (MRI) scan of the cervical spine revealed a heterogeneously enhancing oval, intradural extramedullary (IDEM) mass lesion at the C6-C7 level [Figure 1]. Intraoperatively, the tumor was well encapsulated, soft in consistency and extremely vascular. It had pushed the spinal cord posteriorly and to the left. The C7 rootlets on the right side were entangled within the tumor mass. Complete excision of the anterolaterally situated tumor mass was performed preserving the C7 rootlets. The histopathological examination of the tumor specimen revealed a highly cellular malignant small round cell tumor showing sheet-like proliferation of tumor cells with focal nesting [Figure 2]. Immunohistochemistry revealed strong expression for CD 99 [Figure 3] in tumor cells. They were immunonegative for cytokeratin, EMA, CD 45, TdT, desmin, S100. The FISH test for EWSR1 (Ewing's sarcoma breakpoint region 1) showed break apart signals in 98% of the tumor nuclei indicating the presence of EWSR1 rearrangement [Figure 4]. The above-mentioned findings were classical of Ewing's sarcoma. Following surgery, she was relieved of her symptoms. As part of the screening, a positron emission tomography (PET) CT scan of the whole body and MRI of brain revealed no active disease focus.
Figure 1: Postcontrast image showing IDEM Ewing's sarcoma at C6-7 level showing heterogeneous enhancement

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Figure 2: Hematoxylin and Eosin image. A cellular tumor composed of malignant round cells arranged in a diffuse sheet-like and focal nesting growth patterns (original magnification ×400)

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Figure 3: Immunohistochemistry demonstrates diffuse, strong membrane positivity for CD99 in tumor cells (original magnification ×400)

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Figure 4: FISH Image: Fluorescence in-situ hybridization technique for EWSR1 test showing rearrangement break apart signals in 98% tumor nuclei

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The patient was subsequently treated with six cycles of adjuvant chemotherapy consisting of vincristine, adriamycin, cyclophosphamide, and mesna (VACM protocol). She also received a dose of 40 Gy of radiation therapy to the local tumor bed. At six months of follow-up, she was asymptomatic and had tolerated the chemotherapy very well. A follow-up MRI scan of the cervical spine revealed no evidence of recurrence of tumor [Figure 5].
Figure 5: Follow-up (6 months) postcontrast MRI scan showing complete resection of the C6-7 IDEM Ewing's sarcoma

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Thus, preoperative diagnosis of this rare problem is difficult. However, in cases with short clinical history and rapid progression of clinical symptoms, the probability of Ewing's sarcoma should be considered. Immunohistochemistry and molecular analysis established the diagnosis. Radical to complete excision with adjuvant radiation and chemotherapy is an ideal treatment option to prevent local recurrence and distant spread, thereby prolonging the disease-free survival.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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. 1
    
2.
Kim SW, Shin H. Primary intradural extraosseous Ewing's sarcoma. J Korean Neurosurg Soc 2009;45:179-81.  Back to cited text no. 2
    
3.
Gong HS, Huang QS, Liu GJ, Chen FH, Zhao HB. Cervical primary Ewing's sarcoma in intradural and extramedullary location and skip metastasis to cauda equina. Turk Neurosurg 2015;25:943-7.  Back to cited text no. 3
    
4.
Uesaka T, Amano T, Inamura T, Ikezaki K, Inoha S, Takamatsu M, et al. Intradural, extramedullary spinal Ewing's sarcoma in childhood. J Clin Neurosci 2003;10:122-5.  Back to cited text no. 4
    
5.
Woestenborghs H, Debiec-Rychter M, Renard M, Demaerel P, Van Calenbergh F, Van Gool S, et al. Cytokeratin-positive meningeal peripheral PNET/Ewing's sarcoma of the cervical spinal cord: Diagnostic value of genetic analysis. Int J Surg Pathol 2005;13:93-7.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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