Hidradenoma papilliferum

Hidradenoma papilliferum also called papillary hidradenoma, is benign tumor arising from apocrine sweat glands or anogenital mammary glands (see Figures 2 and 3) ¹. Papillary hidradenoma has been reported primarily to affect Caucasian females, typically during the third to fifth decades of life ². Hidradenoma papilliferum has not been reported prior to puberty ³. The lesions are usually solitary, well-demarcated dermal or subcutaneous papules or nodules covered with normal skin, and are generally less than 10 mm in diameter ⁴. Handa et al. ⁵ reported a case of a large ulcerated perianal hidradenoma papilliferum up to 4 cm in diameter in a young female. Hidradenoma papilliferum tumor is frequently asymptomatic, though may be associated with a itching, burning sensation or pain and bleeding or discharge, especially if there is ulceration of the lesion ⁶. Hidradenoma papilliferum usually occurs in a vulval, perineal or perianal location. When these tumors are not located in the anogenital area, they are termed ectopic hidradenoma papilliferum ⁷. Hidradenoma papilliferum can also occur in male or extragenital areas, this is considered as an ectopic variety ². Ectopic hidradenoma papilliferum, which involves the skin away from anogenital region, is exceedingly rare, and unexpectedly, affects males as well as females ⁸.

Your skin has two types of sweat glands: eccrine and apocrine. Eccrine glands occur over most of your body and open directly onto the surface of your skin. Apocrine glands are usually associated with hair follicles or open into the hair follicle, leading to the surface of the skin. Apocrine sweat glands continuously secrete a fatty sweat into the gland tubule. Emotional stress causes the tubule wall to contract, expelling the fatty secretion to the skin, where local bacteria break it down into odorous fatty acids. Apocrine glands develop in areas abundant in hair follicles, such as on your scalp, armpits and genital regions; the glands are inactive until they are stimulated by hormonal changes in puberty.

Hidradenoma is a benign adnexal neoplasm originating from the apocrine sweat gland ⁹. Hidradenoma is histologically sub-divided into four categories:

1. Tubular,
2. Cystic,
3. Papillary (hidradenoma papilliferum), and
4. Syringocystadenoma papilliferum-like (occurs mostly on the skin of the head and neck and rarely involves the eyelid).

Hidradenoma papilliferum is a benign tumor that requires differentiation from many other diseases such as thrombosed external hemorrhoids, anal abscess, viral warts, and epidermoid carcinoma. Less often, hidradenoma papilliferum may be confused with sebaceous cysts, lipomas, or neurofibromas. In cases of associated pigmentation, hidradenoma papilliferum may also be confused with anal endometriosis, angioma, or melanoma of the anogenital area ¹⁰.

Regardless of the site, the treatment of choice is local excision ⁶. Recurrence of anogenital and ectopic hidradenoma papilliferum is unusual and is typically attributed to incomplete excision of the primary tumor. Malignant transformation is rare. The malignant counterpart is very aggressive, with high rates of recurrence. These have many synonyms, including malignant hidradenoma, hidradenocarcinoma, malignant acrospiroma, acrospirocarcinoma, and clear cell eccrine carcinoma ⁸.

Figure 1. Sweat glands (apocrine and eccrine sweat glands)

Figure 2. Papillary hidradenoma

Footnote: (a) A 7 × 7 mm sized, soft, dome-shaped mass with normal skin colour on the left lateral side of nose (case 1). (b) A 10 × 10 mm sized, slightly erythematous, dome-shaped nodule with central ulceration (case 2). (c) The lumen of the papillary glandular structure was lined with two layers of columnar and cuboidal cell with active decapitation (case 1, haematoxylin and eosin (HE) × 200). (d) Complex anastomosing papillary glandular structure (case 2, HE × 40).

[Source ¹¹ ]

Figure 3. Hidradenoma papilliferum

Footnote: (A) Shows the bluish mass involving the anterior superior-medial aspect of the left orbit (arrow). (B and C) Histopathological slides show papillary glandular pattern with always apparent myoepithelial layer (Haematoxylin and eosin stain, ×40 and ×200, respectively).

[Source ¹² ]

Figure 4. Hidradenoma papilliferum of the anus 

[Source ¹ ]

Hidradenoma papilliferum causes

The cause of hidradenoma papilliferum is unclear. Hidradenoma papilliferum occurs almost exclusively in the female anogenital area; however, there have been a few isolated reports of perianal hidradenoma papilliferum in male patients ¹⁰. Hidradenoma papilliferum tumors were previously believed to originate from apocrine sweat glands; however, some authors believe that rudimentary glandular structures, eccrine sweat glands or ectopic vulvar breast tissue could also be a possible source of the tumor ⁸. In support of this possibility, mammary-like glands have been documented in several nonanogenital areas such as the ears, face, chest, and scalp ¹³ and hidradenoma papilliferum tumors do rarely occur in nonanogenital areas, where they are referred to as ectopic hidradenoma papilliferum. The most common site of ectopic hidradenoma papilliferum is the head and neck ¹³ and in contrast to hidradenoma papilliferum, the incidence of ectopic hidradenoma papilliferum is nearly equal between men and women ¹³.

The specific predilection of hidradenoma papilliferum to the vulva and perianal area could be explained by the abundant apocrine sweat glands, especially the mammary-like glands, which are thought to be the origin of hidradenoma papilliferum ¹². This is because of its similarity to intraductal papilloma of the breast. Moreover, expression of the estrogen receptor in anogenital hidradenoma papilliferum explains the lack of reported cases before puberty and sex predilection ¹².

Hidradenoma papilliferum symptoms

Hidradenoma papilliferum occurs almost exclusively in middle-aged women ¹⁴ and presents as a solitary, circumscribed nodule usually less than 1 cm in diameter that may resemble hemorrhoids ¹⁵. Hidradenoma papilliferum tumor is frequently asymptomatic, though may be associated with a itching, burning sensation or pain and bleeding or discharge, especially if there is ulceration of the lesion ⁶.

Hidradenoma papilliferum diagnosis

Hidradenoma papilliferum diagnosis requires surgical excision and histology. Hidradenoma papilliferum is a well-circumscribed dermal nodule, usually without connection to the overlying epidermis. Microscopically, the lesions are cystic papillary tumors and are composed of proliferation of papillary fronds made of loose fibrous connective tissue lined with a double-layered epithelium. The inner layer is comprised of cuboidal myoepithelial cells and the outer is tall columnar apocrine cells with a pale eosinophilic cytoplasm, which may demonstrate decapitation secretion. Larger fronds may have a fibrous core. Fibrous tissue surrounding the tumor may be compressed to form a pseudocapsule.

Hidradenoma papilliferum treatment

Surgical excision is the treatment of choice regardless of location and size. Surgical excision of the lesion is curative and recurrence is only reported where there is incomplete excision of the primary tumor ⁶.

Hidradenoma papilliferum prognosis

The overall prognosis for both anogenital and ectopic hidradenoma papilliferum is good. Malignant transformation of hidradenoma papilliferum is rare, but there are a few documented cases ¹⁶.

References

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