Deformity of the breast in a premenopausal woman

Nipple retraction or inversion occurs when the nipple is pulled inward. Although the terms retraction and inversion are often used interchangeably, the term retraction should be used when only a slit-like area of the nipple is drawn in, whereas the...

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Nipple retraction or inversion occurs when the nipple is pulled inward. Although the terms retraction and inversion are often used interchangeably, the term retraction should be used when only a slit-like area of the nipple is drawn in, whereas the term inversion should be used when the entire nipple is pulled inward.

Inversion of the nipple can be acquired or congenital, unilateral or bilateral, and umbilicated or invaginated. Umbilicated nipples can be pulled out from beneath the surface of the areola whereas invaginated nipples cannot.¹

Approximately 3% of women have congenitally inverted nipples, of which approximately 87% are bilateral.¹ Differentiating between congenital and acquired nipple inversion is important, as congenital nipple inversion typically has no pathologic implications. Benign retractions tend to be symmetrical, slow developing, and bilateral.² A unilateral, asymmetric, and rapidly progressing inversion should therefore raise concern for more serious underlying pathology such as malignancy.

Other acquired causes include trauma, surgery, infection, and inflammation, such as duct ectasia and periductal mastitis.³ Unlike nipple inversion secondary to malignancy, cases associated with duct ectasia typically do not involve the areola and can be easily everted. Patients tend to be older women with a long history of bilateral, symmetrical inversion and a creamy or greenish nipple discharge. Periductal mastitis tends to occur in younger women who smoke.

 

Normally, a thin layer of contractile muscle in surrounding tissue allows the nipple to protrude centrally through the areola. Congenital nipple inversion can result when the underlying mesenchymal tissue fails to proliferate and project the nipple papilla outward.¹ With malignancy, the balance between forces that pull and protrude the nipple and skin of the breast become skewed. Retraction or inversion is present in approximately 4% of breast cancer patients and occurs most often with a malignancy in the retroareolar region.⁴ Retraction of the skin results from shortening of Cooper ligaments due to tumor invasion. This connective tissue, which extends from the clavicle and branches out to the dermis overlying the breast, normally functions to suspend the breast.

Invasive ductal carcinoma can cause periductal fibrosis resulting in subsequent contraction and nipple inversion.⁵ Due to greater infiltrative behavior, invasive ductal and invasive lobular carcinoma are more frequently associated with nipple inversion.

Thorough physical examination, imaging, and biopsy are necessary for evaluation of new-onset nipple inversion or retraction. Unilateral inversion and asymmetry are more often associated with underlying malignancy. Subtle nipple or skin retraction can be accentuated by certain physical maneuvers, such as having the patient raise her arms above her head. Extensive skin retraction may indicate a neglected tumor or a multicentric or multifocal invasive carcinoma. This is most often seen with lobular carcinomas, as they tend to be multifocal.⁴ If malignancy is suspected, clinicians should look for other signs, including edema, ulceration, nipple discharge, and lymphadenopathy.

Although nipple inversion secondary to malignancy can be due to tumor deep within the breast, it is most often caused by tumors in the retroareolar region. Because the retroareolar tissue is more radiographically dense than the rest of the breast, carcinomas in the region are more difficult to identify on mammography than elsewhere in the breast. In such cases, ultrasound is a useful adjunct to evaluate for a possible subareolar mass not visible on mammography.⁶ Although contrast-enhanced magnetic resonance imaging is not usually part of the workup, it may be valuable if results of both ultrasound and mammography are inconclusive.⁷ Biopsy should be performed if malignancy is suspected.

Patients with nipple inversion or retraction secondary to suspected malignancy should undergo complete evaluation to exclude or confirm breast cancer. If a biopsy confirms malignancy, then determination of hormone receptor status, staging, and any indicated additional imaging studies should be done. Staging is based on the American Joint Committee on Cancer and the International Union for Cancer Control TNM classification. Most patients newly diagnosed with breast cancer have disease confined to the breast with limited or no lymph node involvement. Extensive imaging studies are not routinely performed unless there are signs or symptoms suspicious for locally advanced disease or distant metastasis. Treatment approach depends on such factors as staging, hormone receptor status, and the medical fitness of the patient. Treatment may include surgery, radiation, chemotherapy, endocrine therapy, or ERBB2-directed agents. Patients who have nipple inversion and centrally located tumors may still be eligible for breast-conserving therapy.⁸

The patient in our case was referred to the oncology department and underwent a diagnostic mammogram and ultrasound, followed by a biopsy confirming stage III invasive ductal carcinoma. A whole body positron emission tomography/computed tomography scan did not reveal evidence of distant metastasis. The patient was initially treated with neoadjuvant chemotherapy, followed by total mastectomy and axillary dissection, radiation, and hormonal therapy. She is doing well, with no evidence of recurrence of the disease 2 years after her diagnosis.

Asley E. Turkeltaub, BS, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.

References

1. Park HS, Yoon CH, Kim HJ. The prevalence of congenital inverted nipple. Aesthetic Plast Surg. 1999;23(2):144-146.
2. Whitaker-Worth DL, Carlone V, Susser WS, et al. Dermatologic diseases of the breast and nipple. J Am Acad Dermatol. 2000;43(5 Pt 1):733-751.
3. Da Costa D, Taddese A, Cure ML, et al. Common and unusual diseases of the nipple-areolar complex. Radiographics. 2007;27(suppl 1):S65-S77.
4. Lanyi M. Mammography: Diagnosis and Morphological Analysis. Berlin, Germany: Springer-Verlag; 2003.
5. Powell R. Breast examination. In: Walker H, Hall W, Hurst J, eds. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd ed. Boston, MA: Butterworths; 1990.
6. Giess CS, Keating DM, Osborne MP, et al. Retroareolar breast carcinoma: clinical, imaging, and histopathologic features. Radiology. 1998;207(3):669-673.
7. An H, Kim K, Yu I, et al. Image presentation. The nipple-areolar complex: a pictorial review of common and uncommon conditions. J Ultrasound Med. 2010;29(6):949-962.
8. Haffty BG, Wilson LD, Smith R, et al. Subareolar breast cancer: long-term results with conservative surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 1995;33(1):53-57.