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Cutaneous extrarenal rhabdoid tumor with myogenic differentiation

Background:  The rhabdoid phenotype is characterized by large epithelioid cells with abundant eosinophilic cytoplasm and paranuclear inclusions of intermediate filaments. Although originally described in tumors from pediatric kidneys, the rhabdoid phenotype has since been described in a variety of patient ages and extrarenal sites. Extraordinarily, the rhabdoid phenotype has emerged in cutaneous neoplasms, either as a pure extrarenal rhabdoid tumor or a composite phenotype coupled with another malignancy. Regardless of the clinical setting, the rhabdoid phenotype is uniformly associated with aggressive biological behavior. We report the findings from a rare and very aggressive primary extrarenal rhabdoid tumor of the skin with myogenic differentiation.