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Pathologe 2020 May 26. Epub 2020 May 26.

Pathologisches Institut, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 224, 69120, Heidelberg, Deutschland.

The nipple-areola complex is the origin of various morphologically distinct tumors and tumor-like lesions, which can be delineated from the special structures of the nipple, in particular the intramammary ducts, skin-appendages, and the intramammary stroma. Benign tumors are most frequent and this includes epithelial tumors such as mammary adenoma and syringomatous tumor of the nipple. Less commonly observed are benign mesenchymal tumors such as leiomyoma of the nipple, or tumor-like lesions like pseudo-lymphoma. Read More

Pediatr Ann 2020 Feb;49(2):e66-e70

Benign congenital anomalies are common in neonates. Some of these common benign congenital anomalies include preauricular pits, sacral dimples, supernumerary digits, accessory nipple, and natal teeth. It is important for health care providers who take care of newborns to recognize these benign anomalies so that unnecessary evaluations and investigations are not performed. Read More

J Anat 2020 May 2;236(5):954-962. Epub 2020 Jan 2.

Department of Veterinary Medicine, University of Cambridge, Cambridge, UK.

Supernumerary nipples or teats (polythelia) are congenital accessory structures that may develop at any location along the milk line and have been implicated in the pathogenesis of mastitis. We describe the anatomy and histology of 27 spontaneously occurring supernumerary teats from 16 sheep, delineating two groups of teats - simple and anatomically complex - according to the complexity of the anatomy and microenvironment. Anatomically complex supernumerary teats exhibited significantly increased length and barrel diameter compared with simple supernumerary teats. Read More

Pan Afr Med J 2019 17;34:36. Epub 2019 Sep 17.

Dermatology and Venereology Department, Ibn Sina University Hospital, Rabat, Morocco.

Case Rep Pathol 2019 17;2019:9461815. Epub 2019 Jan 17.

Department of Pathology, Zealand University Hospital, Slagelse, Denmark.

Syringomatous tumour (SyT) is a rare type of benign locally infiltrative tumour with debated origin. Because of the growth pattern, SyT can be mistaken for a malignant tumour, and it is therefore important to keep this diagnosis in mind. This case presents a woman with two supernumerary nipples on each side of the abdomen. Read More

Int J Surg Case Rep 2019 19;55:35-36. Epub 2019 Jan 19.

University of Duhok, College of Medicine, Department of Surgery, Azadi Teaching Hospital, 8 Nakhoshkhana Road, 1014 AM, Duhok City, Iraq. Electronic address:

Introduction: Accessory or supernumerary nipples can be applied when more than 2 breasts present in human beings. Usually they are seen in the embryonic milk line; however, their presence outside this line is extremely rare. A 14-year-old boy presented to the surgical consultation room complaining from a round brown skin lesion over the right shoulder area, there were no symptoms associated with this lesion but the family was worried about it. Read More

Surg Neurol Int 2018 24;9:143. Epub 2018 Jul 24.

Department of Pathology, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India.

Background: Accessory breast, also known as supernumerary breasts, polymastia, or mammae erraticae, is a clinical condition of having an additional breast. Accessory breasts are usually seen along the embryonic milk line, with the majority located in the axilla. Polythelia is the presence of an additional nipple. Read More

Cir Esp 2019 Jan 11;97(1):52. Epub 2018 Jul 11.

Servicio de Cirugía General y del Aparato Digestivo, Unidad de Patología Mamaria, Hospital Universitario Central de Asturias, Oviedo, Asturias, España.

Case Rep Dermatol Med 2018 11;2018:6925105. Epub 2018 Feb 11.

Department of Dermatology, University of Texas McGovern Medical School, Houston, TX, USA.

Lymphangioma circumscriptum is a superficially localized variant of lymphangioma. The characteristic clinical presentation is a "frogspawn" grouping of vesicles or papulovesicles on the proximal limb or limb girdle areas. Though most lymphangiomas develop congenitally, the lymphangioma circumscriptum subtype is known to present in adults. Read More

Ann Dermatol Venereol 2018 Jun - Jul;145(6-7):423-428. Epub 2018 Apr 16.

Cabinet de dermatologie, 16, rue Balard, 75015 Paris, France.

Background: Paget's disease of the breast is rare, even more so when it occurs in a supernumerary breast since diagnosis is delayed due to lack of exploration and ignorance of the pathology of ectopic breast. Based on an original clinical case, we provide a clinical update concerning ectopic breast and ectopic carcinoma, including Paget's disease.

Patients And Methods: A 44-year-old woman had complained of pain in the area of her supernumerary breast for several months. Read More

G Ital Dermatol Venereol 2019 Oct 24;154(5):596-597. Epub 2018 Jan 24.

Unit of Dermatology and Cosmetology, IRCCS University Vita-Salute San Raffaele, Milan, Italy.

Anim Genet 2017 Oct 12;48(5):570-579. Epub 2017 Jul 12.

CAS Key Laboratory of Animal Ecology and Conservation Biology, Institute of Zoology, Chinese Academy of Sciences (CAS), Beijing, 100101, China.

Genome-wide association studies (GWASs) have been widely applied in livestock to identify genes associated with traits of economic interest. Here, we conducted the first GWAS of the supernumerary nipple phenotype in Wadi sheep, a native Chinese sheep breed, based on Ovine Infinium HD SNP BeadChip genotypes in a total of 144 ewes (75 cases with four teats, including two normal and two supernumerary teats, and 69 control cases with two teats). We detected 63 significant SNPs at the chromosome-wise threshold. Read More

Am J Case Rep 2017 Jun 10;18:649-655. Epub 2017 Jun 10.

Department of Pathology, University of Cincinnati, College of Medicine, Cincinnati, OH, USA.

BACKGROUND Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked recessive syndrome characterized by fetal overgrowth. CASE REPORT We present a case of a male infant with SGBS. Abnormal prenatal ultrasound (including congenital diaphragmatic hernia) prompted microarray testing of amniotic fluid cells, which showed deletion on chromosome Xq26. Read More

Rozhl Chir Spring 2017;96(4):179-182

Polymastia is a fairly well-described and not entirely uncommon developmental anomaly in embryogenesis of the breast. It occurs in the milk line, most often in the axila but may also be present outside of this area. Clinical manifestation of polymastia is due to sex hormones, especially during puberty, pregnancy and lactation. Read More

Ann Transl Med 2017 Mar;5(5):106

North Coast Cancer Institute, Lismore, 2480 NSW, Australia.

We describe a 54-year-old man with breast cancer and a BRCA2 mutation who was also found to have bilateral supernumerary nipples. His sister, also with a BRCA2 mutation, was diagnosed with breast cancer in her late forties; she also had bilateral supernumerary nipples. We address the significance of breast cancer arising in breast tissue underlying supernumerary nipples; the known association between supernumerary nipples and genitourinary malignancies/malformations and the possible link between BRCA2 and supernumerary nipple development. Read More

J Clin Diagn Res 2016 Oct 1;10(10):PC18-PC24. Epub 2016 Oct 1.

Assistant Professor, Department of Radiodiagnosis and Imaging, M.M. Institute of Medical Sciences and Research , Mullana, Ambala, Haryana, India .

Introduction: Polymastia and polythelia may be asymptomatic or cause pain, restriction of arm movement, milk discharge, cosmetic problems or anxiety. Cosmesis is the main indication for surgical excision of accessory breasts in axilla. In addition it also confirms the diagnosis and allays the patient's fear of harbouring a malignancy. Read More

J Dairy Sci 2017 Jan 17;100(1):402-411. Epub 2016 Nov 17.

Qualitas AG, Zug 6300, Switzerland.

Supernumerary teats (SNT) are any abnormal teats found on a calf in addition to the usual and functional 4 teats. The presence of SNT has also been termed "hyperthelia" since the end of the 19th century. Supernumerary teats can act as an incubator for bacteria, infecting the whole udder, and can interfere with the positioning of the milking machine, and consequently, have economic relevance. Read More

Breast J 2017 03 31;23(2):246-248. Epub 2016 Oct 31.

Division of Plastic and Reconstructive Surgery, European Institute of Oncology, Milan, Italy.

J Dairy Sci 2016 Nov 17;99(11):8891-8900. Epub 2016 Aug 17.

Génétique, Physiologie et Systèmes d'Elevage (GenPhySE), Université de Toulouse, INRA, INstitut Polytechnique de Toulouse (INPT), Ecole Nationale Vétérnaire de Toulouse (INP-ENVT), Castanet Tolosan, France.

This paper reports a quantitative genetics and genomic analysis of undesired presence of supernumerary teats (SNT) in goats. Supernumerary teats are a problem in goat breeding as they can considerably impede machine milking efficiency, leading to increased milking time and injury. This phenotype has routinely been recorded for the past 15 yr in French Alpine and Saanen goats. Read More

J Med Case Rep 2016 Aug 12;10(1):223. Epub 2016 Aug 12.

Department of Pediatrics, Brody School of Medicine, East Carolina University, 3E-139 Brody Medical Sciences Building, Greenville, NC, 27834, USA.

Background: Pierpont syndrome was first described in 1998 with key characteristics including developmental delay, dysmorphic facial features, fat pads on hands and feet, and feeding difficulties. To date the mechanism of inheritance is unknown. Nine out of ten previously described patients with Pierpont syndrome were boys. Read More

J Pediatr Adolesc Gynecol 2016 Oct 13;29(5):e79-e81. Epub 2016 May 13.

Chief of Pediatric Surgery, Department of Pediatric Surgery, Emergency Children's Hospital, Cluj-Napoca, Romania.

Background: Supernumerary breast or polymastia is a well documented anomaly of the breast, and commonly presents along the embryonic milk line extending between the axilla and groin. However, cases of polymastia have been recorded in the face, vulva, and perineum. The clinical significance of these anomalies include their susceptibility to inflammatory and malignant changes, and their association with other congenital anomalies of the urinary and cardiovascular systems. Read More

Clin Breast Cancer 2016 08 31;16(4):e107-12. Epub 2016 Mar 31.

Department of Human Pathology, University Hospital of Messina, Messina, Italy.

Background: Ectopic breast tissue, which includes both supernumerary breast and aberrant breast tissue, is the most common congenital breast abnormality. Ectopic breast cancers are rare neoplasms that occur in 0.3% to 0. Read More

Clin Dysmorphol 2016 Apr;25(2):84-5

aDepartment of Genetic Medicine, Belfast HSC Trust bCentre for Cancer Research and Cell Biology, Queens University of Belfast, Belfast, UK.

Ann Surg Oncol 2015 Oct 5;22(10):3236-40. Epub 2015 Aug 5.

Department of Surgery, Stanford University School of Medicine, Stanford, CA, USA.

The nipple and areola are pigmented areas of modified skin that connect with the underlying gland of the breast via ducts. The fairly common congenital anomalies of the nipple include inversion, clefts, and supernumerary nipples. The anatomy of the nipple areolar complex is discussed as a foundation to review anatomical variants, and the physiologic development of the nipple, including changes in puberty and pregnancy, as well as the basis of normal physiologic discharge, are addressed. Read More

Ugeskr Laeger 2015 Jun;177(25)

Mammakirurgisk Afdeling, Regionshospitalet Viborg, Heibergs Alle 6, 8800 Viborg.

Two different forms of ectopic breast tissue exist in human beings: supernumerary and aberrant. Both forms are usually seen alongside the milk lines, which extend from the upper limbs to the inguinal region where they give rise to mammary glands, areolas and nipples. Although ectopic- and orthotopic breast tissue are placed in different areas of the body, they still share the same ability to undergo pathological degeneration. Read More

Am J Med Genet A 2015 Sep 29;167A(9):2176-81. Epub 2015 Apr 29.

Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, North Carolina.

The Multiple Congenital Anomalies-Hypotonia-Seizures Syndrome 1 (MCAHS1) has been described in two families to date. We describe a 2-year-old Mexican American boy with the syndrome and additional manifestations not yet reported as part of the phenotype. The patient presented with severe hypotonia, microphallus and left cryptorchidism, and was later diagnosed with epilepsy and severe cortical visual impairment. Read More

Eur Arch Paediatr Dent 2015 Feb 23;16(1):63-6. Epub 2014 Sep 23.

Department of Pedodontics, School of Dentistry, Istanbul Medipol University, 34083, Istanbul, Turkey,

Background: The Simpson-Golabi-Behmel syndrome (SGBS) is an overgrowth condition characterised by macrosomia, mental deficiency, large head, prominent skull sutures, midface deficiency, hypertelorism, broad nose, wide mouth, macroglossia, malocclusion, highly arched palate, and musculoskeletal and limb abnormalities. The aim of this case report is to present clinical and oral findings of an 8-year-old boy who had been diagnosed with SGBS.

Case Report: This patient had supernumerary nipples on the right side, cubitus valgus webbed fingers, scoliosis, umbilical hernia, a coarse face, macrocephaly, hypertelorism, a short broad nose, a wide mouth, a straight facial profile and hearing loss. Read More

Anim Genet 2014 Dec 10;45(6):871-3. Epub 2014 Sep 10.

School of Agricultural, Forest and Food Sciences, Bern University of Applied Sciences, Länggasse 85, 3052, Zollikofen, Switzerland.

Supernumerary teats represent a common abnormality of the bovine udder. A genome-wide association study was performed based on the proportion of the occurrence of supernumerary teats in the daughters of 1097 Holstein bulls. The heritability of caudal supernumerary teats without mammary gland in this study was 0. Read More

Korean J Pediatr 2014 Jun 30;57(6):292-6. Epub 2014 Jun 30.

Department of Pediatrics, Chonnam National University Hospital, Gwangju, Korea.

The deletion of the distal long arm of chromosome 1 is associated with a characteristic facial appearance and a pattern of associated malformations. Characteristic manifestations include a round face with prominent 'cupid's bow' and downturned corners of the mouth, thin vermilion borders of lips, a long upper lip with a smooth philtrum, a short and broad nose, epicanthal folds, apparently low-set ears, micrognathia, microcephaly, abnormal hands and feet, variable cardiac or genital anomalies, moderate to severe mental retardation, and growth retardation. Using fluorescent in situ hybridization (FISH) analysis to map precisely the deletion, we present a case of chromosome 1q44 deletion with craniofacial characteristics, multiple congenital anomalies, and growth and psychomotor retardation. Read More

Colomb Med (Cali) 2014 Jan-Mar;45(1):45-7. Epub 2014 Mar 30.

Doctoral Program in Morphological Sciences, Faculty of Dentistry, Universidad de La Frontera, Temuco, Chile ; Research Center for Biomedical Sciences, Universidad Autónoma de Chile, Temuco, Chile.

Polythelia has been defined as the presence of supernumerary nipples without accessory glandular tissue. Usually, these growths follow imaginary mammary lines running from the armpits to the groin. Although the presence of dental anomalies may occasion only a simple cosmetic problem with specific clinical considerations, the association with familial polythelia has been scarcely reported. Read More

Pediatr Endocrinol Rev 2014 Mar;11(3):290-7

Supernumerary nipples (or polythelia) usually appear along the embryonic milk lines or in other sites including the back, thigh, vulva, neck etc. The frequency of polythelia ranges from 0.2% to 5. Read More

J Appl Anim Welf Sci 2014 ;17(2):178-82

a Department of Farm Building , Agronomy and Environmental Sciences, University of Salamanca , Spain.

The aim of this work was to determine whether the removal of supernumerary teats from dairy sheep when they are born is a useful procedure in the farming routine. Ewes were divided into 3 groups according to the number of teats at milking: ewes who were born with 2 teats; ewes who were born with 4 teats and had the 2 supernumerary nipples cut just after birth; and ewes who were born with 4 teats and did not have nipple amputation performed. Removal of supernumerary teats at lambing produced a significant reduction in milk production during the 2 first milking periods (p < . Read More

Am J Med Genet A 2014 May 29;164A(5):1254-61. Epub 2014 Jan 29.

Department of Pediatrics, University of Jordan, Amman, Jordan.

We describe a 6-month-old female with developmental delay, hypotonia, supernumerary nipples, and distinct craniofacial features. Postnatal chromosome analysis revealed an unbalanced karyotype involving a der (5) and array-CGH defined two unbalanced regions with partial 2.3 Mb deletion of 5q35. Read More

J Breast Health 2014 Jul 1;10(3):181-183. Epub 2014 Jul 1.

Department of Plastic Surgery Ospedale di Circolo e Fondazione Macchi, Plastic Surgery, Varese, Italy.

Polythelia is a rare congenital malformation that occurs in 1-2% of the population. Intra-areolar polythelia is the presence of one or more supernumerary nipples located within the areola. This is extremely rare. Read More

Indian J Hum Genet 2012 Sep;18(3):373-5

Department of Dermatology Leprology Venereology, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh, India.

We present a case of seven nipples in a 32-year-old male patient. The patient had two regular nipples along with five supernumerary nipples. Usually, supernumerary nipples develop along the two vertical "milk lines" which start in the arm pit on each side, run down through the typical nipples, and end at the groin. Read More

Aesthetic Plast Surg 2013 Aug 9;37(4):762-6. Epub 2013 May 9.

Department of Plastic, Reconstructive, and Aesthetic Surgery, Adana Numune Training and Research Hospital, Adana, Turkey.

Unlabelled: Accessory breast tissues including nipples, areolas, and glandular tissue may develop on the chest in addition to two normal breasts. An accessory breast with a complete ductal system, areola, and nipple is termed a "supernumerary breast." Supernumerary nipples are fairly common, but complete supernumerary breasts are rare. Read More

Aesthetic Plast Surg 2013 Aug 26;37(4):746-9. Epub 2013 Apr 26.

Department of Surgery, Breast Unit, IRCCS AOU San Martino-IST, Largo Rosanna Benzi 8, 16132, Genoa, Italy.

Unlabelled: Ectopic breast tissue comes in two forms: supernumerary and aberrant. Despite morphologic differences, ectopic breast tissue presents characteristics analogous to orthotopic breast tissue in terms of function and, most importantly, pathologic degeneration. Data in the literature concerning its precise incidence, the probability of malignant degeneration, and its standardized management are scarce and controversial. Read More

Case Rep Surg 2013 27;2013:947295. Epub 2013 Mar 27.

Surgery Department, Indira Gandhi Medical College and Research Institute, 225, Vazhudavur Road, Kathirkamam, Pondicherry 605009, India.

Introduction. The congenital anomalies of breast, especially the polymastia (supernumerary breast) and polythelia (supernumerary nipple), always do not fail to amuse the clinicians because of their varied presentations, associated renal anomalies, and pathologies arising from them. The axillary polymastia is a variant of ectopic breast tissue (EBT). Read More

Dermatol Online J 2013 Jan 15;19(1). Epub 2013 Jan 15.

University of Texas Medical School at Houston, Houston, TX, USA.

The presence of supernumerary nipples, known as polythelia, is the most common presentation of accessory breast tissue. It is usually considered to be a benign congenital anomaly. However, polythelia may warrant attention for more than mere cosmetic concern because supernumerary nipples have been shown to be associated with an increased risk of genitourinary malignancies. Read More

Semin Plast Surg 2013 Feb;27(1):36-41

Division of Plastic Surgery, University of Montreal, Montreal, Canada.

Poland syndrome is a combination of chest wall deformity and absent or hypoplastic pectoralis muscle and breast associated with shortening and brachysyndactyly of the upper limb. Clinical presentation varies widely; therefore, reconstructive procedures have to be adapted to the deformity, ranging from chest wall stabilization or augmentation, dynamic muscle transfer, nipple and areola repositioning, and breast augmentation using prosthesis or autologous tissue transfer. Other congenital breast anomalies include supernumerary nipple and areola (polythelia) and breast (polymastia), which can generally be found on the embryonic mammary ridge. Read More

Med Arch 2012 ;66(5):348-9

Department of surgery, General Hospital "Dr. Josip Bencevic", Andrije Stampara 42,35000 Slavonski Brod, Croatia.

We present an adolescent boy with unilateral supernumerary breast. It was a V type by Leung Clasification. Despite the average occurence between 0. Read More

Am J Med Genet A 2012 Sep 17;158A(9):2245-9. Epub 2012 Jul 17.

Clinical Genetics Unit-Obstetric and Pediatric Department, Arcispedale Santa Maria Nuova, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy.

The Simpson-Golabi-Behmel syndrome type 1 (SGBS1, OMIM #312870) is an X-linked overgrowth condition comprising abnormal facial appearance, supernumerary nipples, congenital heart defects, polydactyly, fingernail hypoplasia, increased risk of neonatal death and of neoplasia. It is caused by mutation/deletion of the GPC3 gene. We describe a macrosomic 27-week preterm newborn with SGBS1 who presents a novel GPC3 mutation and emphasize the phenotypic aspects which allow a correct diagnosis neonatally in particular the rib malformations, hypoplasia of index finger and of the same fingernail, and 2nd-3rd finger syndactyly. Read More

J Pak Med Assoc 2012 May;62(5):499-500

Department of Plastic and Reconstructive Surgery, Harran University, School of Medicine, Sanliurfa, Turkey.

Polythelia, the presence of supernumerary nipples or nipple-areola complexes, is the most common anomaly of the pediatric breast. Although polythelia is common, intraareolar polythelia (nipple dichotomy) is an extremely rare congenital malformation. Surgical treatment is performed as a prophylaxis against breast cancer which has a higher prevalence in polythelia or polymastia. Read More

Dev Biol 2012 Apr;364(2):149-61

Developmental Biology Program, Institute of Biotechnology, University of Helsinki, Helsinki, Finland.

Mammary glands and hair follicles develop as ectodermal organs sharing common features during embryonic morphogenesis. The molecular signals controlling the initiation and patterning of skin appendages involve the bone morphogenetic proteins and Wnt family members, which are commonly thought to serve as inhibitory and activating cues, respectively. Here, we have examined the role of the Bmp and Wnt pathway modulator Sostdc1 in mammary gland, and hair and vibrissa follicle development using Sostdc1-null mice. Read More

Ann Ital Chir 2012 Mar-Apr;83(2):109-12

Cattedra di Chirurgia Plastica e ricostruttiva, Università degli Studi di Perugia.

Aim: Our study focuses on the approach, management and treatment of patients with polythelia and supernumerary breast and the target is the search for a treatment algorithm for these diseases.

Material Of Study: We considered at 18 patients treated from 2006 to 2011 for breast congenital anomalies in excess. 14 surgical procedures were performed, 8 for supernumerary nipple excision (1 case associated with Poland Syndrome) and 6 for accessory breast ablation. Read More

Tumori 2011 Nov-Dec;97(6):812-4

Department of General Surgery, Norfolk and Norwich University Hospital, Colnet Lane, Norwich, Norfolk, United Kingdom.

Nipple adenoma, a benign tumour of the breast, is a relatively rare occurrence. This report describes an even rarer case of nipple adenoma arising within a supernumerary mammary gland. The presenting symptoms were a lump and throbbing pain in the axilla. Read More

J Dermatol 2012 Oct 14;39(10):873-5. Epub 2011 Dec 14.

Indian J Dermatol Venereol Leprol 2011 Nov-Dec;77(6):730

Cutis 2011 Jun;87(6):300-4

UT Health Science Center, San Antonio, Texas, USA.

Supernumerary breasts and nipples are not uncommon and have familial and syndrome associations. Although usually of only cosmetic concern, hormonal changes and inflammatory or neoplastic conditions that affect primary breast tissue also may occur in areas of ectopic breast tissue. We describe cases of familial functional axillary breasts and primary carcinoma of the breast arising in ectopic axillary breast tissue. Read More