Management of Lesions Behind the Areola (Nipple-Areolar Complex)
For lesions behind the areola, management should include thorough diagnostic imaging followed by appropriate surgical intervention with either breast-conserving surgery or mastectomy depending on the extent of disease, with consideration for nipple-areolar complex sacrifice when necessary to ensure adequate tumor margins. 1
Diagnostic Evaluation
- Complete history, physical examination, and diagnostic breast imaging are essential first steps for evaluating lesions in the nipple-areolar complex 1
- Diagnostic mammography is the standard initial imaging modality, with additional views of the symptomatic breast if recent bilateral screening mammography was performed within 6 months 1
- Ultrasound is a valuable adjunct for evaluating the retroareolar region, especially when mammography is negative 1
- Special techniques such as standoff pad, abundant warm gel, peripheral compression, and rolled-nipple maneuvers improve visualization of retroareolar lesions 1
- MRI is recommended when biopsy of the nipple-areolar complex is positive for Paget's disease to define disease extent and identify additional lesions 1
- MRI provides excellent visualization of dilated ducts and enhancing pathology without requiring duct cannulation 1
- Ductography (galactography) may be useful for identifying and localizing intraductal lesions in patients with pathologic nipple discharge 1
Biopsy Approach
- The skin of the nipple-areolar complex should undergo surgical biopsy that includes full thickness of the epidermis and at least a portion of any clinically involved nipple-areolar complex 1
- Once a suspicious lesion is identified on imaging, image-guided fine-needle aspiration or core-needle biopsy can be performed for histologic diagnosis 1
- Core biopsy is generally superior to fine-needle aspiration for sensitivity, specificity, and correct histologic grading 1
Surgical Management
For Benign or In Situ Lesions
- For lesions in the immediate subareolar area, partial areolar excision with careful approximation can provide adequate tissue removal while maintaining good cosmesis 1
- The primary lesion should be excised with a rim of grossly normal tissue, avoiding excessive sacrifice of breast tissue 1
- For Paget's disease without an associated cancer, breast-conserving surgery should consist of removal of the entire nipple-areolar complex with a negative margin of underlying breast tissue 1
For Malignant Lesions
- Tumors in the subareolar area may require excision of the nipple-areolar complex to ensure adequate tumor margins and to avoid devascularization 1
- When an associated cancer is present elsewhere in the breast with Paget's disease, surgery should include removal of the nipple-areolar complex with a negative margin, and removal of the peripheral cancer using standard breast-conserving technique 1
- Total mastectomy remains a reasonable option for patients with subareolar malignancies, regardless of the absence or presence of an associated cancer 1
- Recent data show that satisfactory local control may be achieved with breast-conserving surgery that includes excision with negative margins of underlying cancer, along with resection of the nipple-areolar complex followed by whole-breast radiation therapy 1
Axillary Management
- Axillary lymph node staging is not necessary when breast-conserving therapy is used to treat Paget's disease with underlying DCIS without evidence of invasive cancer 1
- In the presence of an underlying invasive breast cancer treated with breast-conserving surgery, axillary surgery should be performed according to standard guidelines 1
- In cases treated with total mastectomy, axillary staging is recommended for patients with invasive disease and should be considered for patients with underlying DCIS without evidence of invasive disease 1
Radiation Therapy
- Patients treated with breast conservation should undergo whole-breast radiation 1
- A radiation boost should be considered to the site of the resected nipple-areolar complex and any associated resected cancer site 1
- Extended-field radiation to regional lymph nodes should be used in cases of associated invasive breast cancer with involved lymph nodes 1
- Including the entire nipple-areolar complex in the radiation field has not shown significant complications when treated to a median dose of 64 Gy 2
Special Considerations
- Meticulous hemostasis is critically important to avoid hematoma formation, which can make subsequent physical examination and mammographic interpretation difficult 1
- The surgeon must orient the specimen with sutures, clips, or indelible ink to facilitate pathological assessment of margins 1
- Clips outlining the breast defect may aid in planning radiation therapy and demarcate the tumor bed for future imaging studies 1
- Patients with subareolar breast cancers have shown similar long-term outcomes to those with cancers in other parts of the breast when treated with appropriate therapy 2
Follow-up
- Follow-up should include interval history and physical examination every 6-12 months for 5 years and then annually 1
- Yearly diagnostic mammography is recommended, with the first follow-up mammogram performed 6-12 months after completion of radiation therapy in patients undergoing breast-conserving therapy 1