PRIMARY EMBRYONAL MANDIBULAR RHABDOMYOSARCOMA WITH MULTIPLE METASTASIS IN A YOUNG GIRL :A CASE REPORT

An embryonal rhabdomyosarcoma (ERMS) is a most common , primitive, malignant, soft tissue sarcoma in children under 15 yrs of age. It is considered to result from malignant transformation of primitive mesenchymal cells. Although it has a predominance for head and neck region , it is found less often in oral cavity. Here we report a case of ERMS of mandible in a 13 year old young girl. Clinical and pathologic findings are described , which were confirmed by histochemical and  immunohistochemical  stains.Three cycles of chemotherapy  resulted in over 50% reduction in size of the mass. However, increase in size was noted subsequent to completion of therapy . In nine months, the patient developed multiple metastases, radionuclide bone scan showed increased tracer uptake on left side of mandible in angle, ramus  region, and right pubis region . Patient died  despite chemotherapy .