8405/0 Papillary hidradenoma


Hidradenoma papilliferum


ICD-O-3 topography code: C44

Hidradenoma papilliferum is a benign neoplasm arising from the sweat glands. Most frequently affected is the skin of vulva and perianal regions; rare cases of extragenital or ectopic hidradenoma have been observed. The lesion presents as a slow-growing, skin-coloured, cystic dermal nodule that may ulcerate and bleed. The nodule is composed of tubules and papillary structures arranged in a frond-like pattern. The papillae consist of a central axis of connective tissue covered by two layers of epithelial cells. The fibrous stroma surrounding the cystic cavity may contain neutrophils and is separated from the neighbouring dermis by clefts.
Hidradenoma papilliferum affects adults (age range 20-90 years) and shows a clear predilection for women. Complete excision is curative; malignant transformation is very rare1
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005


Anus and anal canal
ICD-O-3 topography code: C21

A benign neoplasm arising from the sweat glands. It presents as a slow growing cystic nodular lesion most often in the skin of the vulva and the perianal region. It is characterized by the presence of cystic and large papillary structures. The papillary structures contain connective tissue and are covered by two layers of epithelium. Complete excision is curative.

Anal papillary hidradenoma
This rare tumour arises in the perianal apocrine glands, typically in middle-aged women and only exceedingly rarely in men
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Loane J, Kealy WF, Mulcahy G (1998)
Perianal hidradenoma papilliferum occurring in a male: a case report.
Ir J Med Sci 167: 26-7

. It presents as a circumscribed nodule approximately 1 cm in diameter and may resemble a haemorrhoid. Histologically, it consists of a papillary mass with a cyst-like capsule. The papillae are lined by a double layer of epithelial cells, the outer layer being composed of cells containing mucin. The tumour does not express the eccrine marker IKH4, but it must be remembered that adenocarcinoma metastases are also negative for this marker 3
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Ishihara M, Mehregan DR, Hashimoto K, Yotsumoto S, Toi Y, Pietruk T, Mehregan AH, Mehregan DA (1998)
Staining of eccrine and apocrine neoplasms and metastatic adenocarcinoma with IKH-4, a monoclonal antibody specific for the eccrine gland.
J Cutan Pathol 25: 100-5

. Convincing examples of anal apocrine adenocarcinoma have not been published4
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010


ICD-O-3 topography code: C51

In the vulva, papillary hidradenomas arise from specialized anogenital mammary-like glands typically in or adjacent to the intralabial sulcus
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van der Putte SC (1994)
Mammary-like glands of the vulva and their disorders.
Int J Gynecol Pathol 13: 150-60

. The lesions are benign and present as slowly growing, circumscribed cystic nodules. Histologically, papillary hidradenoma is characterized by glandular structures and complex branching papillae with fibrovascular stalks. Glands and papillae are covered by myoepithelial cells and epithelial secretory cells. The surrounding stroma is fibrous.
Papillary hidradenoma occurs rarely in the vulva, but it is the most common benign glandular tumour at this site. The lesion is usually asymptomatic, but prolapse and/or ulceration of the gland may cause bleeding
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003