Abstract

Objective – To report a very rare case of syringocystadenoma papilliferum, describing its clinical presentation, histopathological appearance and surgical management. Case report – An 11-year-old girl presented with a partially lobulated linear plaque on her right lower back present since birth, which was clinically diagnosed at first as linear epidermal nevus. The tumor was surgically removed. The histopathology confirmed the diagnosis of syringocystadenoma papilliferum. Conclusion – Syringocystadenoma papilliferum is a benign adnexal tumor that occurs most often in the head and neck. The tumor has characteristic histological features, and varied and non-distinct clinical findings. Although extremely rare, this lesion should be considered in the differential diagnosis of skin tumors in children.

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