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A unique dermal and subcutaneous botryoid rhabdomyosarcoma admixed with ectopic mature renal tissue, overlying the left lateral lumbosacral region of a 4-1/2-year-old girl is reported. The tumor was devoid of blastemal or immature epithelial elements and nephrogenic rests. The relationship of this lesion to an extrarenal Wilms' tumor with exclusive myogenous differentiation is discussed.
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