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Accessory breast

Accessory breast tissue, also known as polymastia or supernumerary breast, is a congenital characterized by the presence of ectopic glandular outside the normal pectoral , resulting from the incomplete of the embryonic , or "milk line," which extends bilaterally from the to the inguinal area. This condition manifests as additional breast components, which may include glandular , , and , and is classified into eight types based on the components present, ranging from complete breast structures to isolated glandular . The embryological origin traces back to the development of the in the early , where typically only the thoracic portions persist to form the normal breasts, while the remaining segments regress; failure of this regression leads to accessory tissue formation, with no known specific genetic or environmental triggers beyond this developmental process. varies by sex and ethnicity, affecting approximately 2-6% of females and 1-3% of males worldwide, with higher rates reported in Asian populations (up to 5% in women) compared to Caucasians (around 0.6%); about one-third of cases involve multiple accessory sites. The most common location is the , accounting for 60-96% of cases and often bilateral, though rarer ectopic sites include the chest wall, , back, , or even the face; accessory tissue is frequently unnoticed until hormonal changes during , , or cause enlargement and symptoms. Clinically, accessory breast tissue is often asymptomatic but can present with axillary swelling, tenderness, (especially premenstrually), restricted , cosmetic concerns, or secretion during ; in some cases, it may be associated with supernumerary nipples (polythelia), which occur along the same milk line. Complications are uncommon but include the development of benign conditions like or, rarely, into accessory breast cancer, which comprises 0.3-0.6% of all breast carcinomas and requires vigilant monitoring similar to primary tissue. typically involves , with imaging modalities such as ultrasonography (sensitivity ~69%), , or MRI to confirm glandular nature and rule out ; histopathological examination post-excision reveals simple breast tissue in most cases (~83%). Management is conservative for asymptomatic cases, with no intervention required, as the tissue functions similarly to normal breast and may even support lactation; however, surgical excision—via direct incision, liposuction, or a combination—is recommended for symptomatic relief, cosmetic improvement, or suspicion of malignancy, yielding high satisfaction rates and minimal complications (e.g., ~54% complication-free postoperatively). Early detection and awareness are emphasized, particularly in women undergoing routine breast screening, to differentiate accessory tissue from other axillary pathologies like lymphadenopathy.

Anatomy and Embryology

Embryological Origin

During embryonic development, the originate from the , also known as the milk line, which forms as bilateral ectodermal thickenings along the ventral surface of the . This structure appears between the 4th and 6th weeks of gestation, extending continuously from the to the inguinal region, and arises from of epithelial cells in the of the thoracic region. In normal development, the undergoes , with most segments atrophying through a process involving , leaving only paired solid epithelial masses in the pectoral region that develop into the primary thoracic mammary buds. The primary mammary buds then invaginate into the underlying by the end of the first . Failure of this complete results in persistent ectopic mammary tissue along the original line path. The concept of the mammary ridge as a developmental precursor to breast tissue was first described in anatomical observations during the late , with Schultze noting its presence in 1892 and Schmidt naming it the "mammary band" in 1896 based on studies of embryos. These early insights laid the foundation for understanding accessory breast tissue as remnants of incomplete ridge .

Anatomical Variations

Accessory breast tissue, a congenital resulting from the persistence of embryonic remnants, most commonly develops along the milk line extending from the to the . The represents the primary site, occurring in 60-70% of cases, with additional locations including the , lower abdomen, groin, and medial thigh. Other rarer sites outside the typical milk line pathway, such as the face, , or , have been documented but are exceptional. The composition of accessory breast tissue varies widely; it may encompass glandular with ducts and lobules, a , and in complete forms, or limited to isolated glandular elements, alone (polythelia), (polythelia areolaris), or even non-glandular subcutaneous fat and muscle without mammary features. Classifications, such as Kajava's system, delineate these as complete (Class I: full with glandular tissue, , and ) versus incomplete variants lacking one or more components, with glandular-inclusive forms being the primary focus for clinical relevance. Bilateral involvement occurs in approximately one-third of cases, and multiple ectopic sites are rare but possible, potentially leading to symmetric or asymmetric presentations along the milk line. Accurate differentiation from mimicking conditions is crucial; unlike lipomas, which consist solely of without fibroglandular elements, accessory breast contains mammary-specific structures identifiable via or . Similarly, involves inflammatory apocrine gland changes without glandular breast tissue, distinguishing it through absence of hormonal responsiveness and presence of suppuration.

Epidemiology

Prevalence and Incidence

Accessory breast tissue, also known as polymastia, occurs in up to 6% of the population based on clinical, autopsy, and imaging studies. This prevalence is supported by multiple investigations, including those examining routine clinical examinations where rates range from 1% to 5%, and imaging cohorts that report figures approaching 6%. The condition is more prevalent in women, with incidence rates of 2-6%, compared to 1-3% in men. Detection often occurs during periods of hormonal change, such as or , when the tissue may enlarge and become noticeable. Many cases remain undetected due to their nature, leading to potential underreporting in the general . This underdiagnosis is particularly common in individuals without routine or examinations, as the tissue may mimic normal anatomical variations until symptomatic.

Demographic Patterns

Accessory breast tissue, also known as polymastia, shows notable ethnic variations in prevalence. Rates are highest among Asian populations, particularly individuals, where occurrence can reach 5-6%, while lower rates of 0.6-2% are observed in populations. Gender differences influence the detection and presentation of accessory breast tissue, which occurs in both males and females but is more commonly identified in women due to hormonal changes during , , and that cause enlargement. The condition is approximately twice as common in females as in males (male-to-female ratio of ~1:2). Age-related patterns typically involve incidental detection in adulthood, with increased visibility during reproductive years when estrogen-driven growth highlights the tissue. Presentation often aligns with or , peaking in women aged 20-40 years. Geographic and familial clustering of accessory breast tissue is uncommon, though rare genetic links, such as pathogenic variants in the FANCC , have been associated with familial cases across generations. No strong patterns, such as autosomal dominant transmission, have been firmly established, and distributions primarily follow ethnic rather than geographic lines.

Clinical Presentation

Common Manifestations

Accessory breast tissue typically presents as visible or palpable lumps along the embryonic line, which extends from the to the , often resembling normal in and . These lumps are most frequently located in the axillary region, accounting for the majority of cases. In approximately 5-15% of cases, accessory breast tissue includes a and/or complex, which may contribute to noticeable asymmetry in the affected area. The often undergoes enlargement or becomes more prominent during periods of hormonal fluctuation, such as , , or , mirroring the developmental responses of primary tissue. Many instances of accessory tissue are discovered incidentally during routine physical examinations, as the condition is frequently and overlooked until hormonal changes highlight its presence.

Associated Symptoms

Accessory breast tissue, also known as polymastia, is frequently but can cause various discomforts, particularly when influenced by hormonal fluctuations. and tenderness are among the most common symptoms, often presenting as cyclical discomfort that intensifies during premenstrual or menstrual phases, , , or due to the tissue's responsiveness to and progesterone. In a of 52 patients, approximately 23% experienced in the accessory mass specifically during these periods, while daily axillary discomfort exacerbated by hormonal cycles has been reported in case presentations lasting several years. During , engorgement of the tissue may occur, leading to further tenderness in about 6% of affected individuals. Cosmetic concerns significantly impact those with accessory breast, often manifesting as visible or bulging under clothing, particularly in axillary locations, which can cause and affect clothing choices. In the same of 52 patients, over 73% reported uneasiness related to the mass's position, leading to diminished self-confidence and psychological distress such as anxiety. These aesthetic issues may also result in from with garments, contributing to ongoing discomfort. Functional limitations arise primarily in axillary cases, where the tissue can restrict or movement, causing heaviness or vague discomfort during daily activities. Local irritation and potential for secondary issues like sweating-induced moisture in the area may exacerbate these problems, though direct risks are uncommon. Rare acute symptoms include , characterized by inappropriate milk secretion from the accessory nipple, often postpartum or in response to hormonal stimulation, as seen in adolescent and adult cases. Additionally, , including non-lactational or granulomatous forms, can develop in functional accessory tissue, presenting as inflammatory swelling and pain, though such occurrences are infrequent and challenging to diagnose.

Pathophysiology

Developmental Mechanisms

The development of accessory breast tissue, also known as polymastia, arises primarily from the incomplete regression of the embryonic mammary ridge, or milk line, which forms during the fourth to sixth weeks of gestation and extends from the axilla to the inguinal region. In typical human embryogenesis, this ridge involutes by the seventh to tenth weeks, leaving only the pectoral pair of mammary primordia to develop into functional breasts, but persistence of additional segments along the line results in ectopic mammary tissue. Genetic factors, such as an autosomal dominant inheritance pattern with variable expressivity, are implicated in preventing normal regression of these embryonal ridges in utero. At the cellular level, the failure of , or , in the extra mammary primordia is a key mechanism underlying ridge persistence. During normal gestation, apoptotic processes drive the involution of superfluous ridge segments, eliminating primordial breast cell clusters; disruption of this leads to the retention and potential of ectopic glandular . Animal models, such as those in mice, provide valuable insights into these embryological processes, as the formation and selective regression of mammary lines are conserved across mammals. In mice, the embryonic mammary line forms multiple placodes along the ventral axis around embryonic day 10.5, with all but the thoracic pairs undergoing regression through similar apoptotic mechanisms, mirroring the developmental anomalies seen in accessory breast . These models have been instrumental in elucidating the molecular signaling pathways, including Wnt and FGF pathways, that regulate ridge specification and involution.

Histological Features

Accessory breast tissue, also known as supernumerary breast tissue or polymastia, exhibits a microscopic structure that closely resembles that of normal tissue, consisting of glandular elements including ducts and lobules embedded within fibrous stroma and . The ductal structures are lined by epithelial cells, often forming acini that may show secretory activity, particularly during hormonal influences such as or , with surrounding stroma providing structural support similar to the pectoral . In many cases, the tissue includes mature components interspersed among the glandular elements, contributing to its overall composition. Histological variations in accessory breast tissue depend on its developmental class, as described by Kajava's 1915 classification system; for instance, class I features complete glandular with well-formed lobules and ducts, while class IV, the most common form, presents only isolated glandular foci without nipple or areola, potentially showing incomplete or rudimentary acinar development. Some specimens display fibrocystic changes, including cyst formation or apocrine metaplasia, but these are benign and mirror physiological alterations in normal breast . Full acinar structures with secretory are observed in hormonally stimulated cases, whereas others may lack organized lobular architecture due to ectopic location along the milk line. Biopsy findings typically confirm benign mammary glandular , with immunohistochemical positive for markers such as GATA3, mammaglobin, and BRST-2, aiding in from other adnexal structures. Malignant changes, though rare, can occur and include infiltrating ductal , but the remains benign in the absence of such alterations, showing no atypical proliferation unless pathologically induced. This ectopic glandular tissue arises from persistent remnants of the embryonic milk line, but its mature structure parallels that of the primary breast.

Diagnosis

Clinical Assessment

Clinical assessment of accessory breast tissue begins with recognizing scenarios where suspicion arises, such as during or when patients present with cosmetic concerns like visible axillary lumps, which are common manifestations of this condition. Suspected accessory breast is also warranted in cases of cyclical discomfort or swelling along the milk line, particularly during periods of hormonal fluctuation like , , or . History taking is essential and should include inquiries into family history, as accessory breast tissue may exhibit an autosomal-dominant inheritance pattern with incomplete penetrance, though most cases are sporadic. Clinicians should probe for symptoms such as tenderness, pain, or swelling that correlate with hormonal changes, including menstrual cycles, puberty, or pregnancy, as well as any functional issues like restricted arm movement or irritation from clothing. Additional details on the onset and progression of any noticed masses or skin changes help contextualize the presentation. The involves systematic along the embryonic milk line, extending from the to the inguinal region, to identify any swellings, glandular masses, , or areolae that deviate from normal . should be bilateral, as accessory tissue is often symmetric, and focus on characteristics like mobility, tenderness, and relation to hormonal influences, with careful documentation using classifications such as Kajava's to describe the extent of glandular, nipple, or areolar components. Differential diagnosis requires distinguishing accessory breast from other axillary or thoracic masses, including enlarged lymph nodes, lipomas, hernias, sebaceous cysts, or , through careful correlation of history and exam findings. Neoplastic processes, such as tumors, must also be considered and ruled out based on atypical features like rapid growth or fixation, emphasizing the need for a thorough clinical to guide further steps.

Imaging and Confirmation

Ultrasound serves as the first-line imaging modality for evaluating suspected breast tissue, particularly in cases of palpable axillary masses, due to its ability to characterize without . It typically reveals a heterogeneous echotexture resembling normal breast , with interspersed hypoechoic fibroglandular elements and hyperechoic fat lobules, confirming the glandular nature of the . This modality is especially useful in younger patients or during , where it demonstrates high —approaching 100% negative predictive value—for distinguishing benign tissue from other pathologies in symptomatic presentations. However, its overall for detecting or incidental breast is moderate, reported at approximately 69% (95% : 54.9–81.3%), often necessitating correlation with clinical findings. Mammography is employed as a complementary tool, particularly in adult women over 30 years or when is suspected, to assess for dense fibroglandular densities discontinuous from the main or focal asymmetries in the . It may identify accessory as a patch of glandular elements interspersed with fat, but its utility is limited in non-dense breasts or obese patients, where correlation is essential. For more comprehensive evaluation, especially in dense or equivocal cases, (MRI) is recommended, revealing signal intensity and enhancement patterns akin to normal , such as ill-defined subcutaneous masses with homogeneous or variable contrast uptake. Features consistent with benign accessory on these modalities can be classified as category 2, warranting only clinical follow-up without further intervention. If reveals atypical features, such as irregular masses, microcalcifications, or suspicious enhancement, confirmatory is indicated to rule out . or ultrasound-guided core needle is preferred for its minimally invasive nature and high diagnostic accuracy in sampling glandular tissue, providing histopathological confirmation of accessory breast elements adjacent to normal ducts. This approach is particularly crucial in symptomatic cases or when incidental findings prompt concern for rare associated carcinomas.

Management and Treatment

Conservative Approaches

For asymptomatic cases of accessory breast tissue, often referred to as polymastia, is the preferred approach, involving regular self-examinations to monitor for any changes in size, tenderness, or other developments. This strategy is suitable for small, non-palpable masses without associated symptoms, as treatment is generally unnecessary unless complications arise. Supportive care plays a key role in managing mild symptoms such as or discomfort, particularly in the axillary region. Nonsteroidal anti-inflammatory drugs (NSAIDs), like ibuprofen, can provide relief from cyclical tenderness, while well-fitted supportive bras or garments help reduce physical strain and improve comfort during daily activities or movement. Warm or cold compresses applied intermittently may also alleviate localized swelling or , especially during when accessory tissue can become engorged. In such instances, simple analgesics and ice packs are recommended, with cessation of to promote regression without further intervention. Hormonal management may address cyclical linked to menstrual fluctuations, as accessory breast responds similarly to mammary glands. Low-dose oral contraceptive pills can help stabilize levels for - or progesterone-driven symptoms, though evidence is mixed and consultation with a provider is advised. Non-invasive options, such as cryolipolysis (e.g., CoolSculpting) or injections (e.g., Kybella), may be considered for cosmetic reduction of predominantly fatty accessory tissue in cases without significant glandular components. Counseling is essential for patients experiencing psychological distress, such as anxiety or reduced due to cosmetic concerns from visible axillary tissue. Healthcare providers should offer guidance on , including techniques for cosmetic through clothing choices that minimize prominence, alongside referrals for psychological support to address emotional impacts. This holistic approach helps improve quality of life for those opting against more invasive options.

Surgical Interventions

Surgical interventions for accessory breast tissue, also known as polymastia, are primarily indicated for cases involving persistent , recurrent infections, or patient preference for cosmetic improvement. These procedures aim to remove excess glandular, fatty, or tissue while minimizing disruption to surrounding structures, often confirmed via prior clinical or of the tissue type. Excision is the standard surgical approach for symptomatic accessory breast tissue, involving the removal of glandular components and any associated nipples through a curvilinear incision placed within the axillary crease to optimize . The procedure utilizes instruments such as curved and electrocautery for precise dissection, with a small of excised if necessary to address laxity; it is performed under local or general , with careful consideration to avoid impairing function in cases where the tissue is active. A is typically inserted to prevent formation, and the wound is closed in layers using absorbable sutures. Liposuction serves as an alternative or adjunct for predominantly fatty accessory , employing small cannulas (3-4 mm) under tumescent to aspirate excess in a fanning pattern, thereby contouring the area without extensive scarring. This method is suitable for milder cases lacking significant glandular elements or laxity, with an average of 131-208 mL of removed per depending on the extent. Combined and excision may be used for moderate to severe presentations involving both and glandular , enhancing outcomes while reducing postoperative bulk. Postoperative care emphasizes infection prevention through meticulous wound hygiene, including gentle cleansing with soap and water, avoidance of soaking the site, and prophylactic antibiotics if indicated. Patients are advised to wear compression garments to minimize swelling and support healing, with drains removed within 1-2 weeks once output decreases; scar minimization involves sun protection, topical gels, and massage starting after initial healing to reduce . Light arm exercises are encouraged early to prevent stiffness, with full typically achieved in 4-6 weeks.

Complications and Prognosis

Potential Risks

Accessory breast tissue, owing to its histological similarity to normal breast tissue, carries a rare risk of , with developing in the glandular components at an incidence of less than 1% based on reported cases. Primary carcinomas, such as invasive ductal , have been documented in ectopic sites like the , often presenting as palpable lumps and requiring inclusion in differential diagnoses for tumors along the milk line. This low but established risk underscores the potential for neoplastic changes in untreated glandular tissue. Benign conditions such as and can also develop in accessory breast tissue, similar to normal breast tissue. Infections represent another concern for accessory breast tissue with functional glandular elements, particularly during periods of , where or formation can occur. , a chronic inflammatory condition, has been reported in axillary accessory tissue, manifesting as painful masses or that may mimic other pathologies if untreated. Abscesses in this tissue can develop secondary to bacterial entry, leading to localized collections that complicate the clinical picture. Beyond infectious and oncogenic risks, untreated accessory breast tissue may contribute to , especially with hormonal fluctuations causing swelling or tenderness, which in severe cases can limit arm mobility. This persistent discomfort often leads to psychological distress, including anxiety over appearance or fear of underlying disease, particularly in adolescents or during reproductive years. Given these potential risks, screening recommendations for individuals with confirmed functional accessory breast tissue advocate for its inclusion in routine breast examinations, such as and , to monitor for or other changes akin to standard breast tissue protocols. Early detection through such integrated screening is emphasized to mitigate complications from ectopic sites.

Long-Term Outcomes

Accessory breast tissue, also known as polymastia, typically follows a benign course, with most cases remaining stable throughout life in the absence of , particularly when . This stability is attributed to its congenital nature, though the tissue may undergo physiological changes in response to hormonal fluctuations, such as enlargement or tenderness during , , , or . Untreated instances rarely require and do not significantly impact health over the long term. Following , outcomes are generally favorable, with low rates of recurrence reported in short- to medium-term follow-up periods. Surgical excision, the primary for symptomatic cases, effectively alleviates discomfort, cosmetic concerns, and functional limitations, leading to improved in affected individuals. For example, in cohorts undergoing excision, patients experienced relief from and anxiety, with high and minimal complications such as scarring or swelling that resolved conservatively. Conservative approaches, when appropriate, also support stable long-term results without progression in benign presentations. Monitoring recommendations emphasize inclusion of accessory breast tissue in standard protocols, with periodic clinical assessments and imaging such as to detect any changes, mirroring protocols for ectopic glandular structures. Despite these insights, research gaps persist, including limited long-term studies on untreated cohorts, which hinder comprehensive understanding of lifelong stability and optimal surveillance strategies beyond short follow-up durations of 6-12 months. Larger, prospective investigations are needed to address recurrence risks and hormonal influences over decades.

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    Oct 1, 2016 · The incidence of Polymastia (supranumerary breasts) and polythelia (supranumerary nipples) is around 2-6% among the female population [6].