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|Year : 2015 | Volume
| Issue : 3 | Page : 392-394
Dorsal ectopic breast in a case of spinal dysraphism: A rare entity
Vipin K Gupta1, Isha Kapoor1, Rajpal S Punia2, Ashok K Attri1
1 Department of Surgery, Government Medical College and Hospital, Chandigarh, India
2 Department of Pathology, Government Medical College and Hospital, Chandigarh, India
|Date of Web Publication||5-Jun-2015|
Vipin K Gupta
Department of Surgery, Government Medical College and Hospital, Sector 32, Chandigarh
Source of Support: None, Conflict of Interest: None
An ectopic breast, that is present at a distance from the embryonic milk line, is an uncommon condition in the normal population. We describe a case with the presence of a breast in the dorsal region occurring in a patient with meningomyelocele and split cord malformation type I. A dorsally situated breast in a case of split cord malformation has never been reported previously in the literature. This case report highlights that an ectopic breast could be a marker of occult spinal dysraphism. This lesion should be corrected only after appropriate radiological investigations ascertain the underlying pathology.
Keywords: Dorsal breast; ectopic breast; mammae erraticae; meningomyelocele; spinal dysraphism; split cord malformation
|How to cite this article:|
Gupta VK, Kapoor I, Punia RS, Attri AK. Dorsal ectopic breast in a case of spinal dysraphism: A rare entity. Neurol India 2015;63:392-4
| » Introduction|| |
An ectopic breast is a congenital condition in which an abnormal accessory breast tissue is found in addition to the normal breast tissue. It may be present anywhere along the milk line, with the majority of the ectopic breasts being located in the axilla. When present in areas outside the milk line, they are termed as "mammae erraticae." Spinal dysraphism refers to the congenital anomalies characterized by an incomplete fusion of the midline mesenchymal, bony, or neural elements of the spine. We report a case of a well-formed breast in the dorsal region with an underlying split cord malformation type 1. A case of dorsal breast presenting along with a meningomyelocele has been reported once in the literature  but ours is the first case in whom the dorsal breast was co-occurring with an underlying split cord malformation.
| » Case Report|| |
A 16-year-old female patient presented with complaints of a swelling in the lower back which was present since birth and had gradually started increasing in size for the last 3 years. On examination, a soft-to-firm, nontender, midline mass 12 cm × 14 cm was found in the lower dorsal region. The skin overlying the swelling had a distinct nipple with surrounding areola. An aberrant bony mass was appreciated on the left side of the swelling creating a dimple within it. A separate cystic swelling could be felt just below this bone on the left inferior quadrant of the whole lesion [Figure 1]. The breasts in the pectoral region were clinically normal, and there was no enlargement of axillary, cervical or supraclavicular lymph nodes. The magnetic resonance imaging and computed tomography scan revealed the coexistence of spinal dysraphism with diastematomyelia. Posterior elements of D8-L1 vertebra were absent, and there was herniation of the thecal sac and cord at this level with the cord tethered to the overlying subcutaneous fat. A large herniated cerebrospinal fluid sac was present on the left side at D11-12 level associated with a large subcutaneous fat collection extending from the D8-12 level [Figure 2]. Block vertebrae were seen at C6-7, D1-4, and D11-12 levels.
|Figure 1: Physical examination showing a midline dorsal breast with nipple and areola along with a bony mass (arrow) and cyst (arrowhead)|
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|Figure 2: Magnetic resonance imaging and computed tomography scan showing a bony septum splitting the cord with a meningomyelocele sac and fatty breast tissue|
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At surgery, the patient's accessory breast was removed and the cord was untethered. The bony septum was drilled out, and the meningomyelocele sac was excised. Microscopic examination demonstrated numerous terminal duct lobular units and mammary lobules, with a few showing epithelial hyperplasia [Figure 3]a. Histopathology of the meningomyelocele sac showed a cyst with an ependymal lining with papillary infoldings at places [Figure 3]b.
|Figure 3: (a) Photomicrograph showing terminal duct lobular units embedded in a fibrocollagenous stroma (H and E, ×100). (b) Photomicrograph showing a cyst lined by ependymal lining, showing papillary formation (choroid plexus) (H and E, ×40)|
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| » Discussion|| |
Spinal dysraphism includes a spectrum of congenital spinal anomalies that result from failure of dysjunction of the surface ectoderm (forming the skin and dermal appendages) from the neuroectoderm (forming the spinal cord between the 3 rd and 8 th week of gestation). Occult spinal dysraphism (OSD) is characterized by skin-covered lesions without exposed neural tissue. An array of congenital midline lumbosacral cutaneous lesions have been recognized as markers of OSD, namely a port wine stain, hemangioma, hypertrichosis, deviation of the gluteal furrow, lipoma, dimple, and dermal sinus.  Symptoms vary according to the age and the extent of lesion and include low backache, paresthesia, numbness, weakness of lower limbs, scoliosis, congenital talipes equinovarus and bladder incontinence. A significant difference between a pediatric and adult tethered cord is the presence of low backache in adults.  Our case describes the presence of a fully formed dorsal breast in a patient of spinal dysraphism.
Various terms like accessory breasts, polymastia, supernumerary breasts, have been synonymously used to describe an ectopic breast. It is present in 2-6% of the female population.  In ancient times, occurrence of multiple breasts was considered to be a sign of fertility and femininity as they were found in Goddess Artemis and Goddess Astarte.  Embryologically, these arise from an incomplete involution of the milk line, resulting in the formation of accessory mammary tissue from the redundant clusters of ectopic primordial breast cells. Though uncommon, areas outside the milk line like the knee, thigh, buttocks, face, ear and neck have been reported as locations for ectopic breast tissue.  Heterotopic breast tissue found as an epithelial inclusion in the heart has also been reported. 
Multiple theories have been proposed to explain their origin. One of the earliest theories was given by Darwin  who stated that traits which had disappeared generations before, can reappear, since primitive mammals beneath humans on the evolutionary scale had multiple breasts arranged along milk lines. But this could not explain the presence of breasts outside the milk line. Hughes supported the theory of random migration of primordial breast cells away from the mammary crest;  while, Pfeifer et al. postulated the possibility of their development from metaplasia of sweat glands or modified sweat glands.  Schultz attributed them to the displacement of milk lines, laterally or caudally.  Rivero-Celada et al. had demonstrated the presence of multipotent cells in a meningomyelocele leading to the development of carcinoma.  In our case, a fully formed breast was seen, suggesting the likelihood of random migration of primordial cells as described by Hughes.
Kajava classified the accessory breasts into eight types.  In our patient, a complete breast with nipple, areola, and glandular tissue was found and was accordingly classified as Class I type of ectopic breast. Studies have shown that although a supernumerary nipple may be present at birth, an ectopic breast usually develops after hormonal stimulation during puberty, pregnancy or lactation.  Similarly in the present case, the swelling had become prominent during puberty. The clinical differential diagnosis for a midline swelling in the lower back is broad and may prove to be a diagnostic dilemma for the clinician. The diagnosis may include various benign and malignant lesions like a meningocele, meningomyelocele, lipoma, dermoid or a teratoma. It is, therefore, essential to distinguish the swelling from other lesions by physical examination, radiology and fine-needle aspiration cytology/histopathology. Ectopic breasts demonstrate similar hormonal effects as pectoral breasts and are prone to the risk of developing breast diseases like abscess, mastitis, fibroadenoma, fibrocystic disease or breast cancer.  Psychological disturbances in adolescence, and pain and discomfort especially during menstruation, pregnancy, and lactation have been noted.  Furthermore, patients with ectopic breasts have had associated urinary system abnormalities like supernumerary kidneys, polycystic kidney disease, hydronephrosis or ureteric stenosis, possibly due to the parallel development of the mammary structures and the urogenital system.  Our patient, however, had normal kidneys, a fact that was confirmed on ultrasonography.
Although not a frequent finding, a breast present in the dorsal region must alert the clinician regarding the possibility of an underlying spinal dysraphism. It may, therefore, be considered as a cutaneous marker of OSD. It is advised that such masses should be carefully investigated, and surgical excision should only be done after a thorough clinico-radiological evaluation of the patient.
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[Figure 1], [Figure 2], [Figure 3]