What is the appropriate management for a papule (small raised bump) on the tip of the nipple, not on the areola?

Management of Papule on Nipple Tip

A papule on the tip of the nipple requires urgent biopsy to exclude Paget's disease of the breast, which presents as eczematoid changes and is associated with underlying breast malignancy in 80-96% of cases. 1, 2

Immediate Diagnostic Steps

Clinical Evaluation

• Examine for specific features of Paget's disease: eczema-like appearance, erosion, ulceration, bleeding, itching, or burning sensation of the nipple-areola complex 1, 3
• Look for any palpable breast masses or lymphadenopathy, as the presence of a palpable finding increases malignancy risk to 61.5% 1
• Note that Paget's disease diagnosis is frequently delayed for months to years due to its benign appearance, making early biopsy critical 4, 3

Mandatory Tissue Diagnosis

• Perform full-thickness surgical biopsy of the nipple skin immediately - this is the diagnostic standard and must include the full thickness of epidermis and underlying tissue 1
• Do not rely on exfoliative cytology alone, as negative findings do not exclude Paget's disease 3
• Histology will reveal large clear Paget cells in the epidermis if malignant 2

Imaging Workup

Initial Imaging (Age ≥40 years)

• Obtain diagnostic mammography as first-line imaging 5
• Perform targeted ultrasound of the affected breast, as it is more sensitive than mammography for detecting underlying lesions 5
• These studies identify associated breast malignancy present in 80-90% of Paget's disease cases 1

Advanced Imaging

• Order breast MRI to define extent of disease and identify additional lesions not visible on conventional imaging 1
• MRI has 86-100% sensitivity for invasive cancer and helps identify peripheral duct lesions beyond the reach of standard duct excision 1
• MRI is particularly valuable when mammography and ultrasound are unremarkable 1

Treatment Algorithm

If Biopsy Confirms Paget's Disease

Without underlying breast cancer (no mass or imaging abnormality):

• Breast-conserving surgery with complete excision of nipple-areola complex plus negative margin of underlying breast tissue, followed by whole-breast radiation therapy 1
• Total mastectomy remains a reasonable alternative 1
• No axillary staging needed if only DCIS without invasive component 1

With associated breast cancer:

• Remove nipple-areola complex with negative margins AND the peripheral cancer (does not need to be en bloc) 1
• Whole-breast radiation therapy if breast-conserving approach used 1
• Axillary staging required if invasive cancer present 1
• Mastectomy is traditional standard and remains appropriate 1

If Biopsy Shows Intraductal Papilloma

• Complete surgical excision is standard due to 3-14% malignancy upgrade risk 5
• Consider non-operative management only if: non-bloody discharge, benign core biopsy, normal imaging, AND no risk factors (prior ipsilateral breast cancer, BRCA mutation, or atypia) 5

Critical Pitfalls to Avoid

• Never dismiss a persistent nipple lesion as benign dermatitis - any eczematoid change or papule on the nipple that persists requires biopsy 4, 3
• Do not delay biopsy waiting for lesion to resolve, as this is the most common cause of delayed Paget's disease diagnosis 4
• Recognize that 4-20% of Paget's disease cases have no underlying breast malignancy on imaging or even final pathology, but biopsy is still mandatory 6, 7
• In male patients, nipple lesions carry 23-57% malignancy risk and warrant the same aggressive evaluation 1