A rare areolar growth developing late in pregnancy.

Sir,

We present the case of a 28-year-old lactating woman presented to the gynaecology outpatient department with the complaint of a warty growth arising from the areola of her right breast. The growth was slightly painful and interfered with feeding. She noticed the growth during the third trimester of her pregnancy that progressively increased to the present size [Figure 1]. General physical and systemic examination was unremarkable. Examination revealed a unilateral 2 × 3 cm sized, irregular, dark-colored, wrinkled, firm areolar growth just lateral to the nipple having a midline cleft. Small openings were also present at the base of the growth that expressed milk on pressure. Surface temperature was normal and the growth was non tender. The breast showed no other skin changes or nodularity. Left breast was normal with a normal contour and adequate milk discharge. Axillary and supraclavicular lymph nodes were not palpable. Her menarche was at 14 years of age and there was no history of hormonal therapy. There was no family history of similar or other breast anomalies, diseases, or malignancy. Baseline investigations such as hemoglobin, blood sugar level, urine analysis, kidney and liver function test results were normal. The abdomen and pelvic sonography reports were within normal puerperal limits. Cytology of the milky nipple discharge did not reveal any malignant cells. The areolar growth was excised under local anesthesia with a 1 cm margin [Figure 2]. The cut edges were approximated with interrupted sutures and breast support dressing was done. Histopathological examination exhibited elongated rete ridges in the epidermis, pilosebaceous units containing keratin material, and scattered smooth muscle fiberous stroma [Figure 3] along with ductal tissue, suggestive of intra-areolar polythelia also called as nipple dichotomy. Followup visits at 1 and 2 months showed satisfactory healing and lactation.

Figure 1

A 2 × 3 cm size irregular, warty growth arising from the right breast areola

Figure 2

Excised hyperpigmented areolar growth

Figure 3

Histological exam shows elongated rete ridges in the epidermis, pilosebaceous units containing keratin materials, and scattered smooth muscle fiberous stroma and ductal tissue (hematoxylin and eosin stain, ×40)

Polythelia as the name suggests means extrasupernumerary nipple. It is usually found above the milk line [Figure 4], extending from the axilla to the medial end of the inguinal ligament.[1] Polythelia is an anomaly of the pediatric breast; however, intra-areolar polythelia is a rarity[2] with a reported incidence of 0.2%.[3] Nipple dichotomy is present since birth but often becomes evident at the time of puberty, or, as in our case, during pregnancy when hormonal levels increase. The accessory nipples present away from the milk line are called “ectopic supernumerary nipples” and are reported from face, perineum, neck, shoulder, and even the toe.

Figure 4

Embryonic milk line

Polythelia is classified into eight types by Kajava in 1915 [Table 1], based on the presence of glandular tissue, nipple, areola, fat, and hair.[4] Our case had ductal tissue, stroma, and skin, evident on histology as Type II of Kajava's classification. Polythelia has been reported to be associated with central nervous system, gastrointestinal, skeletal, cardiac, and most commonly, renal anomalies.

Table 1

Kajava's classification of polythelia

In humans, mammary glands develop from the mammary ridge during the embryonic phase. These ridges disappear except at the level of the fourth intercostal space on the anterior thorax, where the mammary gland subsequently develops. A few areas fail to regress, which present as accessory nipples (polythelia) or may evolve into a complete mammary gland with fat (polymastia).[5] Intra-areolar nipple often remains unnoticed as a pigmented spot or umbilicated nodule only. Autosomal dominant transmission with incomplete expression is described in familial cases of polythelia.

Differential diagnosis of a case evolving in reproductive age can be pigmented naevus, neurofibroma, dermatofibroma, lipoma, skin tag, papilloma, or wart. Therefore, histology is must in all cases. Clinically, the presence of wrinkled dark thick skin with small cleft in the center, raises the suspicion. Milk-like discharge from the opening of the growth and histology showing ducts within stroma confirmed the diagnosis of polythelia in our case.

Surgery is indicated in symptomatic cases, suspected malignancy, for cosmetic reasons, and as a prophylaxis against breast cancer in future. A very small flat nipple needs follow up, whereas protuberant lesions require excision. One should try to rule out the associated congenital malformations by history, examination, and ultrasonography. Fine-needle aspiration cytology may be tried first if in doubt. Excision when planned is done by the incision made along the lines of Langer. Large-sized nipples require wide excision followed by reconstruction by transpositioning the flaps sutured to one another.