Abstract
Hidradenoma papilliferum occurs mainly in adult women and appears as a solitary small papule or nodule of skin color with cystic appearance involving the vulva.
Histopathologically, the lesion shows a cystic configuration, with a large cavity lined by epithelium, to which are connected clustered and elongated tubules and long papillae. The papillae consist of a central axis of connective tissue lined by an epithelium composed of two layers of cells: a peripheral layer of cuboidal myoepithelial cells and a luminal layer of columnar cells, with evidence of decapitation secretion.
The epithelial cells lining the papillae express various cytokeratins, such as AE1/AE3 and CK5/6, as well as gross cystic disease fluid protein (GCDFP15). Peripheral myoepithelial cells of the double layer lining the papilla express the myoepithelial markers S-100 protein, calponin, and alpha-smooth muscle actin.
Recurrent mutations in PIK3CA and AKT1 are present in most cases.
Hidradenoma papilliferum is a benign neoplasm and simple excision is curative. Exceptional cases of hidradenocarcinoma papilliferum have originated in a pre-existing hidradenoma papilliferum.
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Requena, L., Sangüeza, O. (2017). Hidradenoma Papilliferum. In: Cutaneous Adnexal Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-45704-8_9
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