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Year : 2003  |  Volume : 13  |  Issue : 3  |  Page : 303-305
Primary Ewing sarcoma of skull vault in a child


Gujarat Research and Medical Institute, Usmanpura Imaging Centre Ahmedabad, India

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Keywords: Ewing Sarcoma, Skull vault tumor

How to cite this article:
Gadani S, Mody R P, Solanki R N, Mahajan A. Primary Ewing sarcoma of skull vault in a child. Indian J Radiol Imaging 2003;13:303-5

How to cite this URL:
Gadani S, Mody R P, Solanki R N, Mahajan A. Primary Ewing sarcoma of skull vault in a child. Indian J Radiol Imaging [serial online] 2003 [cited 2020 May 26];13:303-5. Available from: http://www.ijri.org/text.asp?2003/13/3/303/28704

   Introduction Top


We wish to report "Primary Ewing Sarcoma of Skull Vault In A Child." Diagnosed by various radiological and imaging modalities and confirmed by histopathology.


   Case report Top


A 12 year old male child presented with an external swelling in high parietal region in midline for two months .Patient had history of headache with weakness of right upper and lower limbs. There were no signs of raised intracranial tension

Rest of skeletal survey was normal.


   Discussion Top


Ewing sarcoma is a rare highly malignant neoplasm of bone accounting for about 5% of biopsied bone tumours. Common sites of primary Ewing's sarcoma are long bones (47%), pelvis(19%) and ribs (12%). Ewing's sarcoma of skull vault is very rare. It constitutes less than 1% of all the brain tumours. In a recent study done by Paulus et al, of 2500 cases of brain tumours only 9 were sarcomas, out of which only one case was of Ewing's sarcoma. Frontal ,parietal and occipital bones are common sites in the skull. Skull base and facial bones are less commonly involved. Seventy five percent of cases are under the age of 20 years, with peak incidence between 5 to 13 years

Usually patients with primary skull vault Ewing's sarcoma present with pain and swelling .Rarely patient may present with a neurosurgical emergency.Neurological signs and symptoms may be present when tumour is large, compressing or invading the brain parenchyma. these signs and symptoms are according to size of tumor and region of brain parenchyma involved.

On FNAC' Ewing's sarcoma shows a cluster of monomorphic tumor cells with round vesicular nuclei and ill defined vacuolated cytoplasm. Many dissociated cells present with naked nuclei. Demonstration of glycogen in cytoplasm is usually a consistent finding in EWING'S sarcoma. Histologically, Ewing's sarcoma is a highly anaplastic tumour with solidly packed small round cells. Light microscopic, ultrastructural and immunohistochemical features differentiate Ewing's sarcoma from neuroblastoma, lymphoma and rhabdomyosarcoma.

Most common sites for primary Ewing's sarcoma is long bones. Diahysis is more commonly involved, although in 25% cases metaphyses may also be involved. Ewing's sarcoma in long bones present with a permeative pattern of medullary destruction having wide zone of transition ,destruction of cortex ,multilamellar periosteal reaction and presence of soft tissue.

Primary skull lesion present with osteolytic lesion with erosion of inner and outer table associated with soft tissue on plain x-ray.Similar osteolytic lesions in skull vault are noted in eosinophilic granuloma/Hand Schuller Christian syndrome, metastasis, burkitt's lymphoma, fibrous dysplasia, aneurysmal bone cysts, osteoclastoma and giant cell reparative granuloma. Radiologically it is difficult to distinguish them however, they can be distinguished by different pattern of destruction of skull vault, presence of soft tissue, calcification, cystic component and septations. However histopathology is more specific for their differentiation.

Ewings sarcoma of skull has a tendency to create a significant epidural mass and push in to brain . Epidural mass is seen on CT scan .The mass is usually iso to hyperdense on plain scan with heterogenous enhancement on post contrast study. MRI is useful in precise delineation of different tumour components such as extent of bone, dural and parenchymal involvement. Epidural masses rarely invade the dura and invade the brain parenchyma.

Primary Ewing's sarcoma of skull has lesser tendency to metastasize to lung and bones (one of few bone tumors metastasizing to bones).From remote primary sites like pelvis and long bones Ewing's sarcoma may metasize to skull, spine, meninges and brain parenchyma. Extensive workup is indicated in all cases of Ewing's sarcoma of skull to search for extracranial primary sites .Radionuclide scanning is most sensitive in early detection of the primary lesion and metastatic deposits. Metastasis to brain parenchyma and dura is less frequent. CNS parenchymal involvement is more common due to primary bone tumour rather than metastatic disease.

Most of primary Ewing's sarcoma has good prognosis because they can be totally or subtotally excised. However, those arising from skull base may involve vital structures prohibiting surgical excision .In our case tumour caused breach of dura matter however it didn't invade brain parenchyma.

Surgical removal and /or radiotherapy with adjuvant chemotherapy are treatment of choice.Isolated radiotherapy is indicated in inoperable cases .High risk cases should receive combined radiotherapy and surgical treatment, preferably preoperative irradiation to the lesion

Patients with primary skull vault Ewing's sarcoma have excellent prognosis with 2-5 years of disease free survival in 50-80% cases.


   Conclusion Top


Primary Ewing's sarcoma of skull vault is a rare lesion .Patient present with scalp swelling and non specific neurological signs. CT scan and MRI delineate skull vault lesion with associated epidural soft tissue. Histoapathology differentiates it from other primary skull vault lesions. Surgical removal and radiotherapy /chemotherapy is the treatment of choice.Skull vault lesions have good prognosis because they can be totally or subtotally removed[7].

 
   References Top

1.Stoker DJ: Bone tumours (2): Malignant Lesions In R.G. Granger, D.J.Allision ,A.Adam ,A.K Dixon ,Diagnostic radiology(Volume -3) fourth edition Churchill Livingstone. Edinburgh, PP.   Back to cited text no. 1    
2.Dahlin D:Bone Tumours:General aspects and data on 6221 cases .Thierd edition .Springfield ,IL, Charles C Thomas ,1978,278.   Back to cited text no. 2    
3.Jung H , Gutjahr P : Primary sarcomas of skull in children (Author's transl).Laryngo Rhino Otol,39Stuttg , 1975,54:763-771   Back to cited text no. 3    
4.SteinbokP,Flodmark O ,Norman MG , Chan KW , Freyer CJ:Primary Ewing's sarcoma of base of skull.Neurosurgery,1986,19:104-107.  Back to cited text no. 4    
5.Kozlowski K,Cambell J , Mc Alister et al : Rare Primary cranial vault and baseof skull tumours in children :Report of 3O cases and short literature review.Radiol Med, 1991Mar ,81 (3):213-214   Back to cited text no. 5    
6.Mehta Y,Hendrickson FR : CNS involvement in Ewing's sarcoma.Cancer 1974;33:859-862   Back to cited text no. 6    
7.Rueda-Franco F, Lopez-Correla E: Sarcomas in the central nervous system of children .Paediatric Neurosurg 1995;22:49-56  Back to cited text no. 7    

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Correspondence Address:
S Gadani
39 Suryanagar Society, in front of Gandhi Ashram Post Office, Gandhi Ashram, Ahmedabad 380027
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]



 

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    Introduction
    Case report
    Discussion
    Conclusion
    References
    Article Figures

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