Pilomatrixoma is a benign adnexal tumor very common in pediatric age and in young adults that derives from follicular matrix cells. Although clinically it usually presents as a subcutaneous nodule of bluish color less than 3cm in size, multiple clinicopathological variants have been described in the literature. Among these we can find the giant pilomatrixoma, a rare clinical variant that reaches a size greater than or equal to 4cm and can simulate the clinical presentation of a malignant neoplasm.
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