J Cutan Pathol 2007: 34 (Suppl.

1): 9–13 Copyright # Blackwell Munksgaard 2007

doi: 10.1111/j.1600-0560.2006.00713.x
Blackwell Munksgaard. Printed in Singapore Journal of
Cutaneous Pathology

Bilateral aberrant axillary breast tissue

mimicking lipomas: report of a case
and review of the literature
Abstract: A 31 year old Indian woman presented with bilateral Samer H. Ghosn1, Khalil A.
axillary masses that became noticeable with the onset of puberty. The Khatri2 and Jag Bhawan3
masses exhibited similar consistency to the adjacent normal breast 1
Department of Dermatology, American
tissue but lacked an associated nipple complex. The clinical impression University of Beirut Medical Center, Riad El
was lipoma; however, mammography, ultrasonography and skin Solh, Beirut, Lebanon,
2
biopsy revealed ectopic breast tissue. These findings were consistent Skin & Laser Surgery Center of New England,
Chelmsford, MA, Nashua, NH, USA and
with the diagnosis of aberrant breast tissue. A subset of ectopic 3
Dermatopathology Section, Department of
mammary tissue, aberrant breast tissue may constitute a diagnostic Dermatology, Boston University School of
challenge and is often misdiagnosed as lipoma, hidradenitis, follicular Medicine, Boston, MA, USA
cyst, or lymphadenopathy. In addition, some studies have suggested All authors declare no conflicts of interest
that aberrant breast tissue may be at higher risk of malignant
degeneration. Therefore, it’s important that physicians be familiar Samer H. Ghosn, MD, Department of Dermatology,
American University of Beirut Medical Center,
with this condition as this may contribute to the early detection of PO Box 11-0236, Riad El Solh,
ectopic breast cancer. Beirut 1107 2020, Lebanon
Tel: 961-3-731727
Ghosn SH, Khatri KA, Bhawan J. Bilateral aberrant axillary breast Fax: 961-1-745320
e-mail: sg03@aub.edu.lb
tissue mimicking lipomas: report of a case and review of the literature.
J Cutan Pathol 2007; 34 (Suppl. 1): 9–13. # Blackwell Munksgaard 2007. Accepted for publication November 14, 2006

Among the different types of ectopic mammary Case report

tissue (EMT), physicians are mostly familiar with
supernumerary nipple (polythelia). The spectrum of
EMT, however, entails many other entities based on
the presence of glandular tissue, nipple, areola or
a combination of these.1–3 Although the initial
classification by Kajava1 (1915) is currently used
(Table 1), the terminology pertaining to EMT and
its different types constitutes a great source of
confusion when reviewing the literature. A more
practical approach to EMT is to distinguish between
three major categories: polythelia, polymastia/
supernumerary breast and aberrant breast tissue
(ABT) (class IV in Kajava classification).4,5
We herein report a case of bilateral axillary ABT
simulating bilateral axillary lipomas. The character-
istic clinical and epidemiologic features as well as
prognostic and therapeutic implications are dis-
cussed and compared with those of polythelia and
polymastia/supernumerary breast.
A 31-year-old Indian woman presented with
bilateral axillary masses that developed at the age
of 13 years with the onset of puberty. Since then, she
has been experiencing cyclical localized pain and
discomfort within the masses corresponding to her
menstrual cycle but without any associated increase
in size, engorgement or discharge. In addition, she
was unable to wear sleeveless garments due to
embarrassment and frustration with the excess
tissue. The patient is married but denied previous
pregnancies or oral contraceptive intake. Her pre-
vious medical/family history is non-contributory. In
particular, she has no developmental, skeletal or
urologic anomalies.
Physical examination (Fig. 1) showed bilateral
asymmetric axillary soft masses of similar consis-
tency to that of the adjacent normal breast tissue.
The masses measured approximately 4 3 3 cm and
2 3 2 cm on the right and left sides, respectively. No

9
Ghosn et al.
Table 1. Kajava classification of ectopic mammary tissue. By definition,
the nipple consists of lactiferous ducts in a dense fibrous tissue covered
by skin and contains bundles of smooth muscle fibers. This is in contrast
to the areola, which lacks these ducts and contains mostly smooth
muscle bundles and ectopic sebaceous glands
Class I (polymastia) Complete breast(s) with nipple, areola
and glandular tissue
Class II (supernumerary Nipple and glandular tissue but no areola
breast without areola)
Class III (supernumerary Areola and glandular tissue but no nipple
breast without nipple)
Class IV (mamma aberrata) Glandular tissue only
Class V (pseudomamma) Nipple and areola but without
glandular tissue (replaced by fat)
Class VI (polythelia) Nipple only
Class VII (polythelia areolaris) Areola only
Class VIII (polythelia pilosis) Patch of hair only
erythema, warmth, nodularity, tenderness or dis-
charge was appreciated. No nipple, areola or other
lesions overlying the masses or elsewhere along the
milk line could be identified.
Our initial clinical impression was axillary
lipomas. A skin biopsy showed aggregates of normal
mammary glandular tissue erratically distributed in
a fibroadipose stroma (Fig. 2). Mammography and
ultrasonography showed masses that were discon-
tinuous with, but displaying similar characteristics
to, the parenchyma of the adjacent normal breast.
Based on these findings, the diagnosis of bilateral
axillary ABT was made.
Discussion
EMT represents a common developmental anomaly,
with a reported incidence of 5.78%.6 The vast
majority of cases develop along the mammary ridge
also known as the Ômilk line’ which runs bilaterally
from axilla to groin ventrally and from mid-shoulder
Fig. 1. Right axillary breast mass. Note the absence of associated
nipple complex.
10
Fig. 2. Lobules of normal mammary glands with dilated ducts
(hematoxylin-eosin stain; original magnification: 310). Inset shows
normal glandular and ductal tissue (hematoxylin-eosin stain;
original magnification: 320).
to mid-scapula dorsally.7–9 Unusual locations outside
the milk line such as the face,8 posterior thigh,10
perineum11,12 and iliac obturator muscle13 have also
been reported. In normal breast development, the
mammary ridge recedes, leaving only bilateral
mammary tissue at the fourth intercostal space.
Partial regression of this tissue will lead to EMT that
may reveal itself as structures varying from rudimen-
tary to full-grown breast tissue. EMT should be
distinguished from the axillary component of the Ôtail
of Spence’, which is part of and continuous with the
mammary gland proper and not ectopic in nature,7,14
and from the mammary-like glands of the vulva,
which are normally occurring glands in the interlabial
sulcus between the labia minora and labia majora.15
ABT (Table 1) is reported to occur in 0.22% to
6% of the general female population,7,8 occurring
more frequently among Japanese people and less
frequently among whites.5 In most cases, the tissue is
either bilateral or confined to the right side.16,17
Axillary ABT accounts for the vast majority of ABT
cases.18–21 In contrast to polythelia and polymastia/
supernumerary breast which are usually identified at
birth, ABT becomes noticeable and symptomatic
only after hormonal stimulation, usually during
puberty,22 pregnancy4,12,18,23–25 or breast-feeding.26
ABT, however, is not functional because it lacks a
ductal communication with the overlying skin. In
a large series including 69 female patients, the age at
presentation ranged from 13 to 40 years, with a
median of 25 years. The lesions may be asymptom-
atic or associated with swelling and pain because of
engorgement.16 In some instances, the diagnosis is
only made after malignant degeneration or devel-
opment of other pathologic conditions.
Given the lack of associated nipple complex,
and sometimes symptoms, and the late onset of
 Aberrant axillary breast tissue mimicking lipomas
diagnosis, ABT is often misdiagnosed as lipoma,27
hidradenitis,28 follicular cysts and lymphadenopathy,
thus leading to inappropriate therapies. In the large
series of Das et al., the correct diagnosis of ABT was
achieved in only 23.2% of the cases. Other
diagnoses included lipomatous lesion (17.4%),
lymphadenopathy (18.8%) and swellings not other-
wise specified (30.4%).16 Out of 31 cases of isolated
axillary masses evaluated by de Andrade et al., 5
were attributed to ABT.29 For these reasons, ABT
may be more common than appreciated.
When suspected, the diagnosis of ABT may be
confirmed by many modalities. Mammography and
magnetic resonance imaging reveal a discontinuous
mass with, but displaying similar characteristics to, the
parenchyma of the adjacent normal breast.17,30 Fine-
needle aspiration cytology16,24 and histopathology are
alternative useful modalities. Histologically, ABT
consists of islands of unorganized secretory system
without any relationship to the overlying skin. This is
in contrast to polymastia/supernumerary breast
tissue, which consists of an organized ductal system
communicating with the overlying skin.5 Finally, since
ABT is frequently bilateral, diagnosis on one side
should always prompt evaluation of the other side.14
ABT is not only a cosmetic but also a medical
problem. All physiologic processes as well as benign
and malignant tumors of normal breast tissue may
arise in association with ABT.14,19–21,24,29,31–42 Most
reported cases of malignant degeneration have
involved ductal carcinoma (79%),5 but other types
have also been described. Interestingly, two-thirds of
reported primary ectopic breast carcinoma cases
arose within the axillae.36,43 Whether ABT is more
prone to malignant degeneration than breast proper
remains controversial. In a review of 82 cases of
EMT, Marshall et al. found an increased incidence of
cancer in ABT but not in supernumerary breasts.5 It
is suggested that stagnation in the lumina of ABT is
a promoting factor for the development of malig-
nancy.44 Other studies, however, failed to suggest
such increased risk.16,18 In any event, cancers arising
in axillary ABT have a worse outcome, presenting
usually with a more advanced disease and at an
earlier age.4,5,14,43 This may simply reflect late
detection because of the lack of awareness among
physicians and patients.43 Aside from a higher pro-
pensity to involve the lymph nodes, the prognosis of
primary ectopic breast carcinoma is similar to regular
breast carcinoma in the same tumor, node and
metastasis stage.45 Another important consideration
is that foci of axillary ABT may be misinterpreted as
metastatic disease.46,47 Finally, not only ABT but also
polymastia/supernumerary breast may be associated
with pain, difficulty with shoulder range of
motion, irritation from clothing and psychological
disturbances.
ABT should be differentiated from other forms of
EMT. Polythelia (supernumerary nipple) is often
underdiagnosed or misdiagnosed as small benign
nevi. Males and females are equally affected, and
more than 90% of the cases affect the inframam-
mary area.48 A higher incidence is reported among
Native American, African-American, Jewish and
Asian neonates,7,24,49–52 and an autosomal domi-
nant trait with variable penetrance has been
suggested in around 6% of the cases.21,48,53,54
Although not associated with an increased risk of
malignant degeneration, polythelia may be a marker
for underlying urogenital malformations and malig-
nancies. For instance, renal anomalies have been
found in 14.5% of sporadic cases and 32.1% of
familial cases of polythelia.48 A higher incidence of
skeletal anomalies, pyloric stenosis, testicular carci-
noma and aberrant cardiac conduction has also
been reported.14,49,53,55–59 Unlike polythelia, there is
no association between ABT and urogenital/skeletal
anomalies.4,5,60 True polymastia is rare, with
a reported incidence of 0.12%.6 Sixty-five percent
of affected women have a single accessory breast,
while 30% have two additional accessory breasts
present. Rare case of more than four breasts is also
reported.16,23 Some supernumerary breasts are, like
polymastia, functional and capable of lactation.
Rare cases have been reported in men.10,61 For
practical purposes, supernumerary breast can be
approached similarly to polymastia as both con-
ditions appear unassociated with developmental
anomalies or with increased risk of malignant
degeneration.60,62 In addition, both conditions
appear at birth as accessory nipples and exhibit
glandular tissue hyperplasia only during puberty or
pregnancy.60
Prophylactic surgical excision of ABT has been
advocated by some authors as a management
option.18 A recent study, however, found this proce-
dure to be associated with significant morbidity that
may exceed the risk of developing an ectopic breast
cancer.63 Therefore, the current approach is conser-
vative. If the patient is nursing, cessation of breast-
feeding is recommended to allow regression of the
tissue. One anecdotal report, however, describes
a woman who successfully pumped her axillary
breasts to relieve symptoms without discontinuing
nursing.26 Of paramount importance, ABT should
be periodically monitored for potential pathologic
changes, and patients should be instructed to per-
form periodic self-examination to help early detec-
tion of malignant degeneration.
In conclusion, the differential diagnosis of
a lipoma-like mass located along the primitive milk
line and particularly in the axilla should include
ABT, especially when it arises in pubertal girls. We
report this case to familiarize physicians with this
11
Ghosn et al.
condition, as early recognition of ABT could
potentially contribute to early detection of ectopic
breast cancer and affects the overall prognosis and
survival of affected patients.
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