A case of Ewings sarcoma involving mandible, an unusual site.
ES/PNET is relatively uncommon accounting for 6-8% of primary malignant bone tumours.  It is second most common sarcoma in bone & soft tissue in children.  The median age for patient with Ewings family of tumour is 15 years and more than 50% of the patients are adolescents. 
ES/PNET tends to arise in diaphysis, or metaphyseal-diaphyseal portion of long bone, pelvis and ribs are common locations. The skull, vertebrae, scapula & short tubular bone of hands & feet are rarely involved.  Less than 3% of all ES origionates in the maxillofacial region usually involving the mandible. [4,5] Because of the rarity of tumour involving the mandible and misdiagnosed for long time as dental inflammation /cyst the present case is being reported because of the unusual site.
A 15 year old girl visited the department of Dentistry with complaints of pain & swelling in the left side of face. The case was previously attended outside the institute and was treated for long time for dental infection. There was history of swelling & pain in left jaw for 3-4 months. Intraoral examination reveals swelling involving the left mandible.
OPG finding -mandible shows irregular lytic bone destruction with ill-defined margins. The routine hematological investigations (CBC) were with in normal limits except for raised ESR. On histological examination -H & E stained sections showed sheets of uniform small round cells arranged in diffuse pattern with scanty indistinct outline, little cytoplasm & round to oval nuclei. Diagnosis of small round cell tumour with possibility of Ewings sarcoma/PNET was given. The blocks were sent for Immunohistochemical studies showed PAS positivity and CD99 positive. Other markers were also done to rule out other different round cell tumour like malignant lymphoma, metastatic neuroblastoma, small cell osteosarcoma and embryonal rhabdomyosarcoma.
After marker studies the diagnosis of Ewings sarcoma was confirmed. A picture of small round cell tumour with PAS positivity and CD 99 was helpful in finalizing the diagnosis.
[FIGURE 1 OMITTED]
[FIGURE 2 OMITTED]
[FIGURE 3 OMITTED]
Ewings sarcoma is rare in the head and neck region more so in the mandibular area .This case presented with swelling and pain of the left sided jaw and was mistaken and treated for dental inflammation. X ray finding with biopsy helped in diagnosis of the case. This case is presented to emphasize the importance of biopsy in case of jaw swelling as Ewings sarcoma which are very aggressive tumour can rarely present like this so histopathological evaluation should be mandatory.
After marker studies the diagnosis of Ewing's Sarcoma was confirmed .A picture of small round cell with PAS positivity and CD99 was helpful in finalizing the diagnosis. The present case emphasizes the importance of OPG and Biopsy in any persistent jaw swelling as ES a rare aggressive tumour can present like this clinical picture.
Received Date: 02.10.2012
Accepted Date: 02.10.2012
[1.] Ushigome PS, Machinami R, Sorensen PH. Pathology and genetics of tumours of soft tissue and bone. WHO classification of tumours 2002, page 298-300.
[2.] Paulussen M, Frohlich B, Jurgens H. Ewings Tumour: incidence, prognosis and treatment potions. Pediatric drugs .2001; 3(12); 899-913.
[3.] Raney RB, Asmer L, Nerston WA Jr et al; Ewings sarcoma of soft tissue in childhood; a report from intergroup rhabdomyosarcoma study 1972 to 1991. J Clin oncol 1997; l5(2):574-82.
[4.] Talesh KT, Motamedi MHK, Jeihounian M: Ewings sarcoma of the mandibular condyle; Report of a case. J Oreal Maxillofac Surg 2003, 61:1216-1219.
[5.] Potdar GG. Ewings tumour of jaws. Oral surgery 29; 505, 1970.
Rishi Diwan, Manu Mathur, Devendra Mathur
Jhalawar Medical College, Jhalawar, Rajasthan
Correspondence to: Rishi Diwan (email@example.com)
|Printer friendly Cite/link Email Feedback|
|Title Annotation:||CASE REPORT|
|Author:||Diwan, Rishi; Mathur, Manu; Mathur, Devendra|
|Publication:||International Journal of Medical Science and Public Health|
|Date:||Oct 1, 2012|
|Previous Article:||Malignant phylloides tumour with Giant Cells & rhabdomyosarcomatous differentiation: a case report.|
|Next Article:||Pattern of Craniofacial Injuries and socio demographic distribution of Road Traffic Accidents in Bangalore: autopsy study.|