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Breast-nonmalignant
Congenital anomalies
Supernumerary nipples or breasts / accessory glandular tissue
Author: Nat Pernick, M.D.
Editor: Hind Nassar, M.D., Johns Hopkins Medical Institute (see Reviewers page)
Revised: 25 September 2012, last major update February 2010
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.
Definition
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● Persistent epidermal thickenings along milk line from axilla to perineum / vulva due to clusters of primordial breast cells that fail to involute
● Ectopic tissue may be combinations of breast glandular tissue and nipple
Terminology
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● Also called pseudomamma
● Polythelia (3+ nipples) is more common than polymastia (ectopic breast tissue)
Epidemiology
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● Occurs in 2-6% of females and 1-3% of males
Sites
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● Accessory nipples: thorax or abdomen (65%) and axilla (20%) are most common; back, buttock, face, neck are less common
● Accessory glandular tissue: axilla most common
● May be more common on left side in males (Eur J Pediatr 1998;157:821)
Clinical features
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● Usually sporadic, can be familial (6%)
● Often not noticed until pregnancy
● Classified into 8 levels of completeness (Wikipedia, eMedicine)
● May undergo same disease or physiologic processes as other breast tissue, including lactation (J Reprod Med 1994;39:657)
● Associated with renal disease in some studies (7% vs. 1% in controls, Int J Dermatol 1996;35:349, Scand J Urol Nephrol 2008:1 but not all, Pediatr Dermatol 2001;18:291)
● Also associated with hematologic disorders (Pediatr Hematol Oncol 2004;21:461), mitral valve prolapse (Am J Cardiol 2000;86:695)
Case reports
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Disease in supernumerary nipples or breast:
● Carcinoma in vulva (Cancer 1976;38:2570)
● Fibroadenoma (J BUON 2007;12:285)
● Hamartoma (Breast 2006;15:135)
● Metaplastic carcinoma (South Med J 2002;95:462)
● Mucinous adenocarcinoma of vulva (Archives 2002;126:1216)
● Paget’s disease (Virchows Arch 1998;432:289)
● Secretory carcinoma (Archives 2001;125:1372)
Unusual sites:
● Face (J Pediatr Surg 1997;32:1377)
● Foot (Dermatol Online J 2006;12:7)
● Male perineum (Urology 1997;50:122)
Other case reports:
● Occurrence in three generations (Eur J Pediatr 2001;160:375)
Clinical images
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Milk lines
Nipples anterior Nipple on foot Shoulder and other sites
to axilla
Accessory breast tissue of lower abdomen
Treatment
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● Usually no surgery
● Surgery performed for cosmetic reasons or if a lesion has developed in the breast tissue
Micro description (Histopathology)
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● Supernumerary nipple has same features as regular nipple, including hyperpigmentation, slight hyperkeratosis, pilosebaceous structure of Montgomery tubercles, smooth muscle, Toker cells (J Cutan Pathol 2003;30:256), possibly breast lobules and ducts
Micro images
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Normal breast tissue in Fibroadenoma in axillary Secretory carcinoma in axilla
axilla of 12 year old girl supernumerary breast
Mucinous adenocarcinoma in vulva
Differential diagnosis
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● Lentigos and melanocytic nevi: have melanocytic nests; no pilosebaceous structure of Montgomery tubercles, no smooth muscle organized as with nipple, no Toker cells, no breast glandular tissue
Additional references
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End of Breast-nonmalignant > Congenital anomalies > Supernumerary nipples / breasts
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