Rhabdomyosarcoma (RMS) is a fast growing, highly aggressive malignant tumor, consisting of cells derived from the progenitor cells of myoblasts called satellite cells that exhibit a profound tendency for myogenesis. Accounts for about 5-6% of childhood cancers, occurring in the first 10 years of life. Annual incidence of RMS ranges from 5 to 8/million in Asian and Caucasian children. About 35% of RMS arises in the head and neck region, and are classified as parameningeal (PM), orbital, nonorbital and non-PM forms. PM tumors carry the worst prognosis, and are associated with a high rate of recurrence, and generalized metastases through the hematogenic and/or lymphatic routes. We present a case of 20-year-old male with an exuberant, rapidly increasing swelling of the floor of the mouth. Computed tomography scan, blood investigation, histopathological examination and desmin immuno histo chemistry staining marker, were aids that led to a definitive diagnosis.
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