Rhabdomyosarcoma (RMS) is a malignant neoplasm of primitive mesenchyme exhibiting skeletal muscle differentiation. Intraoral Rhabdomyosarcomas are rare. We report here a case of pleomorphic Rhabdomyosarcoma of oral cavity in a 10-year-old child. The patient was diagnosed clinically and radiographically and planned for surgery. The clinical specimen was sent for histopathological studies . Definitive diagnosis is made by microscopic analysis and other auxiliary techniques such as immunohistochemistry, electron microscopy, cytogenetic analysis and molecular biology. Clinical, histological and immunohistochemical aspects suggests the diagnosis of rhabdomyosarcoma. Although rare, Rhabdomyosarcoma should be included in the differential diagnosis of intra-oral lesions, especially in children. This case illustrates clinical, histopathological aspects and diagnostic difficulties of Rhabdomyosarcoma. Taken together, we conclude that in children, any fast growing swelling should be carefully examined with a high degree of suspicion of this entity.Timely diagnosis and multidisciplinary treatment approach may improve the patient's survival.
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