By Loukas, Marios Clarke, Pamela; Tubbs, R Shane
The presence of accessory breast tissue such as extra nipples (polythelia) and extra breast (polymastia) 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. ACCESSORY BREAST TISSUE is a relatively common occurrence that has a high incidence of being misdiagnosed in clinical medicine. Various diagnoses of such structures include lipoma, lymphatic malformation, lymphadenitis, and sebaceous cysts.1,2 Clinically, cases of accessory breast tissue may be asymptomatic 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 anxiety over the possibility of malignancy. Potential accessory breast tissue in a patient merits further investigation by the clinician, as this tissue has the ability to undergo all the pathological changes that are characteristic of the normal breast and the presence of ectopic 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 multiple breasts (Fig. 1). During the centuries of witchhunts, 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 on the lateral aspect of her left thigh that enlarged during puberty and produced milk when she became pregnant. The famous woodcut showing Venire’s children 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 polythelia than do men. Instances of ectopic breast tissue are higher in blacks Americans, white Americans, Native 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 accessory 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, including nipples, areola, and glandular breast tissue, can be found in addition to the two normally developed breasts on the chest. Most commonly, this tissue develops along the embryonic mammary ridge that extends from the axilla to the groin (Fig. 3). Incomplete regression of this ridge during embryologie formation gives rise to ectopic breast tissue. Aberrant breast tissue has been reported to arise from extra sites, including 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 another posits that accessory breast tissue may occur anywhere apocrine sweat glands are found.17
FIG. 1. The statue of polymastia of Artemis.
In 1915, Kajava2, 19 classified the expression of accessory 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 extension of the axillary tail of Spence.
Accessory breast tissue is not usually identified at a young age. The tissue frequently becomes symptomatic during menarche, pregnancy, or lactation as it responds 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.
FIG. 2. One of the most referred cases of accessory breast tissue from 1827 that involves Therese Ventre of Marseilles. Ventre 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 Venire’s children nursing from her normally positioned right breast and left thigh breast.
Patients with accessory breast tissue may also be more prone to other congenital anomalies. Although there is some dispute over the findings, research indicates 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 diagnosed 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 pulmonary hypertension, cardiomyopathy arising from myocardial infarction, and systemic hypertension are notably related to polythelia.26,27 Ectopic breast tissue in patients is an important cutaneous indicator of conduction system abnormalities, such as bundle branch block or third degree heart block.26
FIG. 3. A woman with two axillary breasts.
Polythelia is a well-established clinical finding in Simpson- Golabi-Behmel Syndrome. Simpson-GolabiBehmel 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 glands.28 The most common presumptive diagnoses include lipoma, lymphadenopathy, hidradenitis, sebaceous cyst, vascular malformation, and malignancy.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 phylloides, extramammary Paget’s disease, and papillary carcinoma have all been reported in accessory mammary tissue.28,35
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.
TABLE 1. Renal Anomalies Found in Correlation with Accessory Breast Tissue
Sentinel node biopsy is effective in accurately determining 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, including 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 ambiguity, a pathological concern, or if the patient wishes to have it removed as a cosmetic hindrance.17 The literature 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 consideration by researchers and the creation of innovative clinical treatment. REFERENCES
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MARIOS LOUKAS, M.D., PH.D.*[dagger] PAMELA CLARKE,* R. SHANE TUBBS[double dagger]
From the * Department of Anatomical Sciences, St. George’s University, School of Medicine, Grenada, West
Indies; the [dagger] Department of Education and Development, Harvard Medical School, Boston, Massachusetts;
and the [double dagger] Department of Cell Biology and section of Pediatric Neurosurgery, University of Alabama,
Address correspondence and reprint requests to Dr. Marios Loukas, M.D., Ph.D., Associate Professor, Department of Anatomical Sciences, St. George’s University, School of Medicine, True Blue Campus, Grenada, West Indies.
Copyright Southeastern Surgical Congress May 2007
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