Micrograph of a papillary hidradenoma with papillary structures; fragmented overlying epidermis is at the top of the image. H&E stain.
Specialty
Gynecology, dermatology
Symptoms
Mass in the genital or other area
Treatment
Surgical removal
Prognosis
Excellent
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. PMID 27742746. S2CID 39876856.
^ 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. PMID 28648934.
^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. PMID 23991738.
^ abcBirge O, Bakır MS, Karadag C, Eldarova Z, Simsek T (April 2021). "Hidradenoma papilliferum of the hymen: a case report". Journal of Medical Case Reports. 15 (1): 162. doi:10.1186/s13256-021-02786-6. PMC 8042849. PMID 33845900.
^Arora K, El-Zaatari ZM, Schwartz MR, Ro JY (August 2020). "Lesions of anogenital mammary-like glands: Four cases including novel pathologic and immunohistochemical observations". Annals of Diagnostic Pathology. 47: 151551. doi:10.1016/j.anndiagpath.2020.151551. PMID 32592993. S2CID 220130115.
^Chauhan H, Tandon P, Potlia I, Jain E (2020). "Rare and unusual occurrence of ectopic hidradenoma papilliferum in maxillofacial region". Journal of Oral and Maxillofacial Pathology. 24 (3): 554–557. doi:10.4103/jomfp.JOMFP_126_20. PMC 8083398. PMID 33967496.
^ abKonstantinova AM, Michal M, Kacerovska D, Spagnolo DV, Stewart CJ, Kutzner H, Zelger B, Plaza JA, Denisjuk N, Hejda V, Shelekhova K, Bisceglia M, Danis D, Zamecnik M, Kerl K, Guenova E, Kazakov DV (August 2016). "Hidradenoma Papilliferum: A Clinicopathologic Study of 264 Tumors From 261 Patients, With Emphasis on Mammary-Type Alterations" (PDF). The American Journal of Dermatopathology. 38 (8): 598–607. doi:10.1097/DAD.0000000000000495. PMID 26863059. S2CID 30597376.
^ abTosti G, Salvini C, Barisani A, Grazi R, Pirola S, Cantisani C, Vaccari S, Madnani N (December 2020). "Vulval hidradenoma papilliferum: a clinical and dermoscopic study". Clinical and Experimental Dermatology. 45 (8): 1035–1039. doi:10.1111/ced.14254. PMID 32356582. S2CID 218475319.
^Pfarr N, Sinn HP, Klauschen F, Flechtenmacher C, Bockmayr M, Ridinger K, von Winterfeld M, Warth A, Lorenz K, Budczies J, Penzel R, Lennerz JK, Endris V, Weichert W, Stenzinger A (February 2016). "Mutations in genes encoding PI3K-AKT and MAPK signaling define anogenital papillary hidradenoma". Genes, Chromosomes & Cancer. 55 (2): 113–9. doi:10.1002/gcc.22315. PMID 26493284. S2CID 31091082.
^Kim GY, Solanki MH, Guo R (August 2021). "Vulvar apocrine hidradenocarcinoma arising in a hidradenoma papilliferum-A case report". Journal of Cutaneous Pathology. 48 (8): 1085–1087. doi:10.1111/cup.14033. PMID 33908057. S2CID 233428232.
^Robbins and Cotran Pathologic Basis of Disease, 7th edition. The Female Genital Tract chapter 22, pg. 1067.
^ abPatel S, Lambert WC, Behbanani S, Espinal-Mariotte JD, Lee P (2020). "Hidradenoma Papilliferum: Everyone Else's Diagnosis". Indian Journal of Dermatology. 65 (2): 151–153. doi:10.4103/ijd.IJD_256_18. PMC 7059462. PMID 32180606.
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