Özet:

Rhabdomyosarcoma is the most common malignancy of soft tissue in childhood and adolescence. It has been traditionally classified into 3 main subtypes: embryonal, alveolar and pleomorphic. However a new, rare subtype called Spindle cell/sclerosing Rhabdomyosarcoma has been defined recently and classified as the fourth variant by the World Health Organization in 2013. We report a case of a 4 year-old girl who had a solid mass in her lower lip with a size of 1cm. In the histopathologic examination the lesion was predominantly composed of mitotic active spindle-shaped cells with hyperchromatic nuclei in a sclerotic stroma and some rhabdomyoblastic cells with eosinophilic cross-striated cytoplasm. All of these cells were stained positive with Vimentin, Desmin, Myogenin, CD 99 and CD 56. Therefore the lesion was diagnosed as Spindle cell/sclerosing pathologists and the clinicians that this rare entity should be remembered in the differential diagnosis of spindle-cell tumors in childhood

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