What are apocrine gland tumors?

Updated: Oct 07, 2019
  • Author: Guy J Petruzzelli, MD, PhD, MBA, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Hidradenoma papilliferum

Hidradenoma papilliferum is a benign adenoma of the apocrine gland that occurs in the vulva and perineum of adult women. This lesion is a benign papillary tumor with cystic characteristics. Although considered a skin lesion of the anogenital region in women, case reports have described these adenomas outside the anogenital region in both sexes. The nonanogenital growths have been termed ectopic hidradenoma papilliferums and occur frequently (60%) in the head and neck region. [118] Simple surgical excision is definitive treatment. [118, 119]

Apocrine adenocarcinoma (extramammary Paget disease)

Clinical presentation

Paget disease commonly refers to the malignant infiltration of breast cancer (typically ductal carcinoma) into the apocrine glands of the areola of the nipple. Extramammary Paget disease is a rare tumor that occurs in the anogenital region that is often, but not necessarily always, associated with adenocarcinoma of the apocrine gland. [120] Cases have been reported that demonstrate Paget disease arising from transitional cell carcinoma of the bladder, adenocarcinoma of the rectum, and squamous carcinoma of the cervix.

Histologically, the thickened epidermis is infiltrated by Paget cells, which are large pale cells with clear cytoplasm. If the cells are derived from mucin-secreting epithelium, then staining with Alcian blue dye can reveal the deposition of mucin within the cytoplasm. This presence of mucin is diagnostically important. In contrast, in extramammary Paget disease from SCC, mucin is absent and is not a distinguishing factor. Therefore, diagnostic difficulties arise because of the complexity in distinguishing atypical melanocytic hyperplasia from squamous carcinoma in situ. [121, 122] Definitive diagnosis requires immunohistochemical staining of the biopsy sample.


For minimally invasive extramammary Paget disease, treatment options include surgical excision with clear margins or Mohs microscopy surgery. [123, 120]  Vigilance is needed after removal of the lesion due to high rates of recurrence.

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