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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 13  |  Issue : 1  |  Page : 23-25
Radiological appearance of Ewing's sarcoma of the mandible


Mohan Dai Oswal Cancer Hospital, G.T. Road, Sherpur, By Pass, Ludhiana, India

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Keywords: Ewings Sarcoma

How to cite this article:
Singh J P, Garg L, Shrimali R, Gupta V. Radiological appearance of Ewing's sarcoma of the mandible. Indian J Radiol Imaging 2003;13:23-5

How to cite this URL:
Singh J P, Garg L, Shrimali R, Gupta V. Radiological appearance of Ewing's sarcoma of the mandible. Indian J Radiol Imaging [serial online] 2003 [cited 2019 Oct 23];13:23-5. Available from: http://www.ijri.org/text.asp?2003/13/1/23/28619

   Introduction Top


The sixth most common malignant bone tumor, Ewing's sarcoma (ES) is a disease of the young [1]. James Ewing originally described it in 1921 as arising from undifferentiated osseous mesenchymal cells; however recent studies suggest that Ewing's tumor might be neuroectodermally derived with various degrees of differentiation of the primitive neural tissue [2]. Ewing's sarcoma is a malignant tumor, which is most commonly found in the long bones and pelvis, it rarely is found in the bones of the face [3]. We report here a case of ES of the mandible in a 20-year-old female because of its rare site and difference from typical radiological appearances elsewhere.


   Case history Top


A 20-year-old female presented to the radiotherapy department with swelling of the mandible on right side. There was no pain or fever and the swelling was not tender. Her Hb was 13.9 gm% other blood profiles were normal. Plain radiograph mandible AP & lateral views [Figure - 1][Figure - 2] showed a lytic permeative destruction of the right ramus of the mandible with soft tissue swelling (not appreciable in these pictures). Axial CT sections were taken [Figure - 3][Figure - 4] and they showed a large soft tissue attenuation lesion arising from the medullary cavity of the ramus of the mandible on the right side. There was expansion of the cortex with the soft tissue mass extending medially into the oral cavity and superficially into the overlying soft tissue structures. On MRI the lesion was hypointense on T1W [Figure - 5] and hyperintense on T2W [Figure - 6] sequences. The hypointense lesion contrasted well with the high signal from the unaffected fatty marrow on the T1W sequence. Bone scan showed increased uptake of radiotracer - hot margin with a central cold area was seen. On imaging the differential diagnosis of lymphoma, lytic type of osteogenic sarcoma, plasmacytoma and histiocytosis X were considered. Metastasis, Ewing's sarcoma and rhabdomyosarcoma are rare in the mandible but were considered among the possibilities; On HPE, the features were suggestive of Ewing's sarcoma. Immunohistochemical CD45 TRO lymphoma test was negative. In view of the histopathological and immunohistochemical features being consistent with Ewing's sarcoma, the patient was given radiotherapy followed by chemotherapy.


   Discussion Top


Ewing's sarcoma is an unusual disease comprising about 4-6% of all primary bone tumors. Although it has been reported as occurring at all ages, by far the majority develops within the first two decades of life. In the second decade, it is the most common primary neoplasm of bone after osteosarcoma. The usual site of occurrence is in the diaphysis of long bones, less often in ribs, pelvis and vertebrae [4]. Involvement of the head and neck in Ewing's sarcoma is very unusual, accounting for approximately 1% to 4% of cases [5]. Males are affected somewhat more commonly than females; the ratio is 3:2. Unlike osteosarcoma, Ewing's sarcoma has a predilection for whites; blacks and Asians are uncommonly affected [1]

Generally the patient presents with pain and local swelling, dilated veins, hyperthermia, anemia, increased erythrocyte sedimentation rate and leukocytosis. A history of previous trauma is present in many reported cases [5]. The typical radiographic appearance of Ewing's sarcoma is a permeative destruction of bone associated with a large soft tissue mass. Lesions can cause a periosteal reaction with the so-called characteristic lamination (onion skinning) or vertical spiculation in as many as one half of the cases. Despite this, onion skinning is reported to be either uncommon or excessively rare in the jaw. The common radiologic features reported in Ewing's sarcoma of the jaw are as follows: a poorly defined permeative lesion with or without associated sun-ray spicules of the periosteal bone, localized honeycomb appearance, cortical erosion, a soft tissue mass next to bony destruction, displacement or destruction of unerupted tooth follicles, advanced eruption and widespread periodontal ligament space [2].

The extent of both intraosseous and adjacent soft tissue involvement can be underestimated if scintigraphy or CT is not used. In our case CT revealed both the soft tissue mass and the intraosseous origin of the tumor. Magnetic resonance imaging is the imaging modality of choice to evaluate the extent of the primary lesion, to monitor the response to neoadjuvant chemotherapy and to follow up non-resected Ewing's sarcoma. Bone scintigraphy is necessary to detect skeletal metastasis, and 201-thalium scanning has been shown to be sensitive in the monitoring of the treatment response [6].

The radiologic differential interpretation of Ewing's sarcoma of the mandible consists of osteogenic sarcoma, neuroblastoma, lymphosarcoma, histiocytosis X, rhabdomyosarcoma, osteomyelitis and metastatic carcinoma. The presence of a large soft tissue mass aided differentiation of Ewing's tumor from osteomyelitis and eosinophilic granuloma [2]. The age of the patient ruled out the possibility of neuroblastoma, which is common in less than 5 years age group. However, radiology is not a totally reliable guide to diagnosis and histopathological examination is necessary to confirm the nature of the tumor.

Facial sites of Ewing's sarcoma carry a better prognosis than long bone or pelvic location, since facial sites are diagnosed earlier [7]. Cure rates of Ewing's sarcoma have improved from 10% to 75% with the use of a combination of local therapy and aggressive multi-drug chemotherapy, to decrease both the incidence of local disease recurrence and the development of pulmonary and skeletal metastases. In Ewing's sarcoma of the mandible, complete surgical resection is preferred to irradiation for local control in those instances when surgery can be performed with minimal loss of function and disfigurement (8). To conclude, although the classical feature i.e. onion peel type periosteal reaction described in Ewing's sarcoma elsewhere, may not be present, a lytic expansile lesion of the mandible with a soft tissue mass and intramedullary origin should suggest in young age the possibility of Ewing's sarcoma as a differential diagnosis.

 
   References Top

1.Eggli KD, Quiogue T, Moser RP. Ewing's sarcoma: RCNA 1993 Mar; 31(2): 325-37.   Back to cited text no. 1    
2.Yalein S, Turoglu T, Ozdamar S et al. Ewing's tumor of the mandible: Oral surgery oral med oral pathol 1993; 76: 362-7.  Back to cited text no. 2    
3.Fielding AF, Lindemeyer R, Wood-Hartman MJ. Ewing's sarcoma of the mandible: combined approach to treatment: J Clin Pediatr Dent 2002 summer; 26(4): 409-12.  Back to cited text no. 3    
4.Hardy P, Gibbs AR. Ewing's sarcoma of mandible: British Journal of Oral and Maxillofacial Surgery 1984; 22: 287-94.  Back to cited text no. 4    
5.Fonseca AS, Mezzalira R, Crespo AN, Bortoleto AE Jr, Paschoal Jr. Ewing's sarcoma of the head & neck: Sao Paula Med J 2000 Nov; 118(6): 198-200.  Back to cited text no. 5    
6.Henk CB, Gramp PS, Wiesbauer P et al. Ewing sarcoma. Diagnostic imaging: Radiologe 1998 Jun; 38(6): 509-22.  Back to cited text no. 6    
7.Langman AW, Kaplan MJ, Matthay K. Ewing's sarcoma of the mandible: Otolaryngol Head Neck Surg 1989 Jan; 100(1): 74-7.  Back to cited text no. 7    

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Correspondence Address:
J P Singh
Mohan Dai Oswal Cancer Hospital, G.T. Road, Sherpur, By Pass, Ludhiana
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]

This article has been cited by
1 Paediatric head and neck imaging
Micallef, C., Saunders, D.E.
Imaging. 2007; 19(1): 104-121
[Pubmed]



 

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    Introduction
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