A papillary hidradenoma, also termed hidradenoma papilliferum or mammary-like gland adenoma of the vulva,[1] is a rare, but nonetheless most common benign tumor that occurs in and between anal and genital regions (i.e. anogenital area) of females.[2] These hidradenomas are sharply circumscribed, nodular tumors that usually develop in women's anogenital area (particularly the vulva[3]) but uncommonly occur in other sites in women and men.[4] Papillary hidradenomas that develop outside of the anogenital region are termed ecctopic papillary hidradenomas or ectopic hidradenoma papilliferums.[5]
Anogenital papillary hidradenomas are regarded as tumors that form in anogenital mammary–like glands (MLAGs); MLAGs are a type of apocrine gland.[2][6] MLAGs were once classified as abnormally located breast tissue glands (see accessory breast tissue@ https://doi.org/10.53347/rID-11125) but are now considered to be normal components of the anogenital region. Microscopically, papillary hidradenomas often resemble certain types of breast tumors.[2] Ectopic papillary hidradenomas are thought to be tumors of apocrine glands which have an as yet unclear relationship to MLAGs.[7]
Typically, papillary hidradenomas are solitary, slow-growing, small tumors that may have been present for months to many years before the time of diagnosis.[8] The tumors are usually symptomless but may be intermittently or constantly painful, become ulcerated, and exhibit minor levels of bleeding.[9] The pain resulting from these tumors may be simple tenderness, more or less constant usually mild pain,[4][9] or, in females, dyspareunia, i.e. pain during and just after sexual intercourse.[4]
Rarely, papillary hidradenomas have progressed to what appear to be 1)in situ malignancies (i.e. malignancies that have neither metastasized nor invaded beyond the layer of tissue where they arose); 2) locally invasive malignancies;[10][11] or 3) complex tumors intermixed with other malignancies such as extramammary Paget disease, melanoma, and squamous cell carcinoma.[8] Furthermore, benign papillary hidradenoma tumors may be confused clinically with cancerous tumors[12] and recent studies have indicated that other types of tumors have often been mistakenly diagnosed as papillary hidradenomas.[13] Papillary hidradenomas, particularly ectopic papillary hidradenomas, require further study to better define the criteria for their diagnosis.[13]
^Goto K, Maeda D, Kudo-Asabe Y, Hibiya T, Hayashi A, Fukayama M, Ohashi K, Goto A (May 2017). "PIK3CA and AKT1 mutations in hidradenoma papilliferum". Journal of Clinical Pathology. 70 (5): 424–427. doi:10.1136/jclinpath-2016-204003. PMID27742746. S2CID39876856.
^ abcKonstantinova AM, Vanecek T, Martinek P, Kyrpychova L, Spagnolo DV, Stewart CR, Portelli F, Michal M, Kazakov DV (June 2017). "Molecular alterations in lesions of anogenital mammary-like glands and their mammary counterparts including hidradenoma papilliferum, intraductal papilloma, fibroadenoma and phyllodes tumor". Annals of Diagnostic Pathology. 28: 12–18. doi:10.1016/j.anndiagpath.2017.02.004. PMID28648934.
^Baker GM, Selim MA, Hoang MP (September 2013). "Vulvar adnexal lesions: a 32-year, single-institution review from Massachusetts General Hospital". Archives of Pathology & Laboratory Medicine. 137 (9): 1237–46. doi:10.5858/arpa.2012-0434-OA. PMID23991738.