Accessory Breasts: A Historical and

Current Perspective
MARIOS LOUKAS, M.D., PH.D.*† PAMELA CLARKE,* R. SHANE TUBBS‡

From the *Department of Anatomical Sciences, St. George’s University, School of Medicine, Grenada, West
Indies; the †Department of Education and Development, Harvard Medical School, Boston, Massachusetts;
and the ‡Department of Cell Biology and Section of Pediatric Neurosurgery, University of Alabama,
Birmingham, Alabama

The presence of accessory breast tissue such as extra nipples (polythelia) and extra breast (poly-
mastia) is relatively common, with a high incidence of being misdiagnosed in clinical medicine.
Although polythelia is congenital in origin and is identifiable at childhood, polymastia may not
be evident until the influence of sex hormones during puberty. In this article, we present a review
of the literature concerning the historical background of accessory breasts, their incidence, their
misdiagnoses, and their association with other syndromes and diseases. Finally, we present the
common treatment options available today for such conditions.

is a relatively common
A CCESSORY BREAST TISSUE
occurrence that has a high incidence of being mis-
diagnosed in clinical medicine. Various diagnoses of
such structures include lipoma, lymphatic malforma-
tion, lymphadenitis, and sebaceous cysts.1, 2 Clini-
cally, cases of accessory breast tissue may be asymp-
tomatic or patients may report discomfort during
menstruation, pain, discharge, and restriction of arm
movement. Many patients may wish to have ectopic
tissue excised for cosmetic reasons or to relieve anxi-
ety over the possibility of malignancy. Potential ac-
cessory breast tissue in a patient merits further inves-
tigation by the clinician, as this tissue has the ability to
undergo all the pathological changes that are charac-
teristic of the normal breast and the presence of ec-
topic breast tissue may indicate underlying congenital
anomalies.
In ancient times, multiple breasts were associated
with fertility. The goddess Artemis is one of the most
well known of the female deities endowed with mul-
tiple breasts (Fig. 1). During the centuries of witch-
hunts, supernumerary breasts and nipples were
deemed Devil’s marks.3 Men and women found with
this accessory breast tissue were often tortured and
killed. One of the most referred to cases of accessory
breast tissue is from 1827 and involves Therese Ventre
of Marseilles, France.4 Ventre had an accessory breast
Address correspondence and reprint requests to Dr. Marios
Loukas, M.D., Ph.D., Associate Professor, Department of Ana-
tomical Sciences, St. George’s University, School of Medicine,
True Blue Campus, Grenada, West Indies.
on the lateral aspect of her left thigh that enlarged
during puberty and produced milk when she became
pregnant. The famous woodcut showing Ventre’s chil-
dren nursing from both of her normally positioned
right breast and left thigh breast is frequently depicted
(Fig. 2).
The prevalence of accessory breast tissue has been
shown to be dependent on a few factors, including
gender, ethnicity, geographical area, and inheritance.
Overall, the occurrence averages between 0.22 per
cent and 6 per cent of the general population.5, 6
Women report a higher rate of polymastia and poly-
thelia than do men. Instances of ectopic breast tissue
are higher in blacks Americans, white Americans, Na-
tive Americans, Japanese, Israeli Jews, and Arabs.7–9
Studies on white European children show a very low
frequency of 0.22 per cent.9 Most instances of acces-
sory breast tissue is sporadic, however, familial cases
have been described in up to 10 per cent of the affected
population. Family pedigrees have mapped the two
most common methods of inheritance to be autosomal
dominant with incomplete penetrance and X-linked
dominance.10–12 Each of these modes of inheritance
shows variability in their phenotypic expression
among generations.
Any combination of accessory breast tissue, includ-
ing nipples, areola, and glandular breast tissue, can be
found in addition to the two normally developed
breasts on the chest. Most commonly, this tissue de-
velops along the embryonic mammary ridge that ex-
tends from the axilla to the groin (Fig. 3). Incomplete
regression of this ridge during embryologic formation
gives rise to ectopic breast tissue. Aberrant breast tis-

525
526 THE AMERICAN SURGEON
FIG. 1. The statue of polymastia of Artemis.
sue has been reported to arise from extra sites, includ-
ing the face, posterior neck, chest, buttock, vulva, hip,
shoulder, posterior and/or lateral thigh, perineum, as
well as the midback (Fig. 4).13–18 Several theories
have been developed to account for breast tissue found
outside the embryonic milk line. One theory suggests
that the milk ridges become displaced, whereas an-
other posits that accessory breast tissue may occur
anywhere apocrine sweat glands are found.17
In 1915, Kajava2, 19 classified the expression of ac-
cessory breast tissue into eight categories still in use
today: complete supernumerary nipple (SN) with
nipple, areola, and glandular breast tissue, which is
known as polymastia; SN with nipple and glandular
tissue without areola; SN with areola and glandular
tissue without nipple; aberrant glandular tissue only;
SN with nipple, areola, and pseudomamma, which is
fat tissue that replaces the glandular tissue; SN with
nipple only, which is known as polythelia; SN with
areola only, which is known as polythelia areolaris;
and a patch of hair only, which is known as polythelia
pilosa. The most common type of accessory breast
tissue is polythelia. Axillary accessory breast tissue is
found in 60 per cent to 70 per cent of all affected
patients. This tissue is separate from the direct exten-
sion of the axillary tail of Spence.
Accessory breast tissue is not usually identified at a
young age. The tissue frequently becomes symptom-
May 2007 Vol. 73
FIG. 2. One of the most referred cases of accessory breast
tissue from 1827 that involves Therese Ventre of Marseilles. Ven-
tre had an accessory breast on the lateral aspect of her left thigh
that enlarged during puberty and produced milk when she became
pregnant. The famous woodcut showing Ventre’s children nursing
from her normally positioned right breast and left thigh breast.
atic during menarche, pregnancy, or lactation as it re-
sponds to normal fluctuating levels of hormones.20
Ectopic breast tissue has been known to change size
cyclically with menstruation, to increase in size during
pregnancy, and to lactate while nursing.
Patients with accessory breast tissue may also be
more prone to other congenital anomalies. Although
there is some dispute over the findings, research indi-
cates a correlation between ectopic breast tissue and
urogenital abnormalities (Table 1). 21 Urogenital
anomalies occur in 1 per cent to 2 per cent of the
general population, whereas an estimated 14.5 per cent
of patients with accessory breast tissue have been di-
agnosed by ultrasound with kidney and/or urinary tract
abnormalities.22–24 This high association has led some
researchers to suggest that there may be a common
supernumerary breast tissue/renal field defect.22, 25
Accessory breast tissue has also been associated
with underlying cardiovascular disorders, although the
relation between the two has yet to be definitively
established. Congenital heart anomalies with pulmo-
nary hypertension, cardiomyopathy arising from myo-
cardial infarction, and systemic hypertension are no-
tably related to polythelia.26, 27 Ectopic breast tissue in
patients is an important cutaneous indicator of con-
No. 5 ACCESSORY BREASTS
FIG. 3. A woman with two axillary breasts.
duction system abnormalities, such as bundle branch
block or third degree heart block.26
Polythelia is a well-established clinical finding in
Simpson-Golabi-Behmel Syndrome. Simpson-Golabi-
Behmel Syndrome is an X-linked recessive disorder
characterized by pre- or postnatal overgrowth, facial
dysmorphic features, polythelia, heart malformations,
cleft palate, and postaxial polydactyly.9
Accessory breast tissue has not been conclusively
linked to renal or cardiovascular disorders, however,
many clinicians choose to follow up affected patients
with renal ultrasound and a full cardiovascular workup
for screening of any congenital or acquired disorders.
Misdiagnosis of accessory breast tissue is common,
especially if the tissue is in close proximity to sweat
FIG. 4. A case of an accessory breast found at the anatomy
department during a routine dissection. The accessory breast was
located to the left side of the cadaver superior to the inguinal
ligament.
⭈ Loukas et al. 527
TABLE 1. Renal Anomalies Found in Correlation with
Accessory Breast Tissue
Brown and Schwartz22 Hypoplastic kidney, bladder
diverticulum, hypotonic bladder
Urbani and Betti25 Polycystic kidney disease,
unilateral renal agenesis, familial
renal cysts, congenital stenosis
of pyeloureteral joint
Leung17 Pyloric stenosis, renal cysts
Meggyessy24 Hydronephrosis, Wilms tumor,
hypoplastic kidney, duplication
of kidney and ureter
Mehes and Pinter40 Hydronephrosis, polycystic
kidneys, renal dysplasia,
duplication of kidney and ureter
Mehes41 Bilateral hydronephrosis
glands.28 The most common presumptive diagnoses
include lipoma, lymphadenopathy, hidradenitis, seba-
ceous cyst, vascular malformation, and malig-
nancy.28, 29 The diagnosis of accessory breast tissue is
supported by the initial appearance during pregnancy
or by a description of cyclical changes in the tissue
during the menstrual period. If doubt exists as to the
nature of the tissue, mammography, needle biopsy, or
surgical biopsy of the area should be undertaken.30–32
Exact diagnosis is crucial, as breast carcinoma can
invade these aberrant areas.33, 34 Ductal carcinoma is
the most frequent subtype of primary ectopic breast
cancer. Medullary breast cancer, cystosarcoma phyl-
loides, extramammary Paget’s disease, and papillary
carcinoma have all been reported in accessory mam-
mary tissue.28, 35
Sentinel node biopsy is effective in accurately de-
termining the staging of cancer in accessory breast
tissue.36 Because the lymphatic drainage from ectopic
breast tissue is unclear, blind dissection of areas such
as the axilla can result in considerable morbidity, in-
cluding intercostobrachial nerve injury, incomplete
excision of the accessory tissue, poor wound healing,
and lymphoadenoma of the arm.29, 36–38
There is no need to specifically treat accessory
breast tissue unless there is some diagnostic ambigu-
ity, a pathological concern, or if the patient wishes to
have it removed as a cosmetic hindrance.17 The litera-
ture reports that the majority of patients want removal
of the tissue for cosmetic reasons.35, 37 Patients should
be completely informed as to the inherent risks of
surgical excision and should be offered liposuction as
an alternative if this option is feasible.35, 39 The study
of accessory mammary tissue and its relationship to
other congenital anomalies warrants increased consid-
eration by researchers and the creation of innovative
clinical treatment.
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