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Supernumerary Nipples
Supernumerary Nipples and Other Forms of Accessory Mammary Tissue
Synonyms
  • Accessory nipples
  • Polythelia
  • Pseudomamma
  • Supernumerary breasts
  • Polymastia
Supernumerary nipples and other forms of accessory mammary tissue are remnants of the embryologic mammary ridges or 'milk lines'; symmetric ectodermal thickenings that extend from the anterior axillary fold to the upper medial thigh (Fig. 64.16). Normally, there is further development in the pectoral region to form the nipples, while the remainder of the milk line regresses; focal areas of persistence result in accessory mammary tissue. Supernumerary nipples, the most common type of accessory mammary tissue, are found in 1-6% of the population44. They usually represent a sporadic developmental anomaly, although approximately 10% of cases are familial. The incidence of supernumerary nipples is similar in men and women. However, accessory mammary tissue in women often becomes prominent at puberty or during pregnancy, and is then more easily recognized. Conversely, changes in supernumerary nipples may actually represent an early cutaneous sign of pregnancy.
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    Figure 64.16 'Milk lines'. Supernumerary nipples and other forms of accessory mammary tissue represent focal remnants of the embryologic mammary ridges that extend from the anterior axillary fold to the upper medial thigh bilaterally.

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    Figure 64.17 Supernumerary nipple. Note the supernumerary nipple with a surrounding areola in a typical location on the inframammary chest. Courtesy of Jean L Bolognia, M.D.

Supernumerary nipples are most commonly seen on the inframammary chest, but they may be located anywhere along the milk lines and occasionally in other sites such as the upper back, shoulder, posterior thigh, face, or neck. In addition, supernumerary nipples sometimes overlie the primary nipple or areola. Lesions are most often single, but they may be multiple and/or bilateral. Supernumerary nipples typically present as a small, soft, pink or brown papule, either with a surrounding areola (Fig. 64.17) or without (referred to as pseudomamma and polythelia, respectively). An areola also can develop in the absence of a nipple (polythelia areolaris), and ectopic glandular breast tissue can be found either associated with a nipple and/or areola (supernumerary breasts, polymastia) or alone. In the latter situation, tissue is most often located in the axilla or vulva, and it may demonstrate all of the functional changes of normal breasts, from tenderness and swelling related to the menstrual cycle to lactation through pores in the overlying skin. Of note, a patch of hair located along the milk lines may be a marker of underlying mammary tissue (polythelia pilosa)45.
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A number of other abnormalities have been described in patients with supernumerary nipples. The association between supernumerary nipples and malformations of the kidneys and urinary tract is controversial. Although earlier reports supported the existence of a 'supernumerary nipple/renal field defect', several recent controlled studies failed to confirm this association46. However, a few studies have found supernumerary nipples to be significantly more common in patients with urogenital or renal malignancies than in control groups. An increased incidence of predominantly ipsilateral Becker's nevi has also been noted in patients with supernumerary nipples. Supernumerary nipples are found in several multiple congenital anomaly syndromes, including Simpson-Golabi-Behmel syndrome (pre- and postnatal overgrowth, coarse faces, embryonal tumors and congenital cardiac, renal and/or skeletal anomalies), cleft lip/palate-ectodermal dysplasia syndrome, and tricho-odonto-onychial dysplasia ( www.ncbi.nlm.nih.gov/OMIM www.ncbi.nlm.nih.gov/OMIM).
Histologic features of accessory mammary tissue are similar to those of normal breasts. For example, supernumerary nipples show acanthosis, pilosebaceous structures, smooth muscle and, in many cases, mammary glands. Fine needle aspiration cytology can be helpful for evaluation of an axillary or vulvar mass suspected to represent breast tissue, although an incisional or excisional biopsy may be required for definitive diagnosis. Ectopic glandular breast tissue can develop any of the disorders that occur in normally positioned breasts, including fibrocystic changes, mastitis, fibroadenomas, and carcinoma. Although such tissue does not have an increased malignant potential compared with the normal breast and prophylactic excision for cancer prevention is not required, routine screening should be performed with periodic physical examination and radiologic studies such as mammography or ultrasonography. Complete surgical excision is the treatment of choice for accessory mammary tissue that is symptomatic or cosmetically undesirable. Lastly, most authors do not recommend imaging studies to exclude an associated urinary tract malformation in asymptomatic individuals with accessory mammary tissue as an isolated anomaly

 

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