Primary cutaneous adnexal carcinomas are exceedingly rare in pediatric patients. A 12-year-old girl presented to the dermatology clinic with a congenital subcutaneous nodule on the left buttock. The patient underwent wide local excision, and histopathologic findings demonstrated large nodular aggregates of atypical basaloid cells with a sieve-like pattern. Immunohistochemical stains were positive for p63, AE1/AE3, β-catenin, and EMA. CK7, CK20, CD117, SOX-10, and Ber-Ep4 were negative. To the best of our knowledge upon review of the literature, we describe a new entity of an adnexal carcinoma with a cribriform pattern in a pediatric patient. Although there was no clinical evidence of recurrence at follow-up, the biologic potential of this tumor is unknown.
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