Pagets Disease of the Nipple

Right sided Paget's Disease

Left side normal nipple and areola

Hyperkeratosis right nipple and areola

A 15yr old girl presented to the clinic with complaints of itching in the nipple area of left breast for the past 2 years. She was shy to reveal her distress to her parents. She is otherwise healthy and has no family history of breast cancer. On examination, the whole of the nipple and areolar region was covered with hyperkeratotic plaques, but no oozing. No underlying lump was palpable. No axillary lymph node enlargement was seen. Plaques were sent off for cytological examination. She was advised to have a mammography and also to undergo a breast biopsy tomorrow.


Sir James Paget first described Paget disease (PD) of the breast in 1874. He reported a chronic eczematous disease on the skin of the nipple and the areola in 15 women, with an associated intraductal carcinoma of the underlying mammary gland.

 It is now widely accepted that mammary PD is always associated with an underlying carcinoma of the breast. By thorough histologic examination, Muir documented intraepidermal extension of malignant ductal epithelial cells through the lactiferous ducts and ductules into the epidermis (epidermotropism).^[2] The findings are the basis of the epidermotropic theory of mammary PD.





                                                                          nearly 100% of mammary PD cases are associated with an underlying carcinoma, either in situ
(intraductal, 10%) or infiltrating cancer (90%)
 In female patients with mammary PD, ages range from 24 to 84 years, with a mean age at diagnosis of 55 years; the average age range is 53-59 years. The average patient age is 5-10 years older for patients with mammary PD than for individuals with breast carcinoma.
 Mammary PD occurs almost exclusively in females; PD of the male breast is extremely rare


Survival is related to the presence or the absence of a palpable breast tumor. When present, the prognosis is the poorest. In one study, 31 (62%) of 50 patients with mammary PD presented with a detectable breast mass.
One half (50%) of patients with PD presenting with a palpable breast mass have associated axillary lymph node metastasis. Two thirds of patients with axillary node metastasis were reported to have a palpable breast mass, whereas one third of patients with axillary metastasis did not have a palpable mass.

Mastectomy is the standard treatment of mammary Paget disease. Conservative treatment with preservation of the nipple-areola complex results in a higher rate of recurrence than treatment by mastectomy.

Mammography changes include

• Subareolar microcalcifications (helpful in evaluating and locating clinically occult, nonpalpable underlying breast carcinoma)
• Architectural distortion
• Thickening of the nipple and the areola (reflecting edema)
• Nipple changes (in a minority of patients)
   
   
  Statistical evidence suggests that in the setting of negative mammography findings, magnetic resonance imaging (MRI) of the involved breast can detect otherwise occult PD and thus facilitate treatment planning for patients with PD.
   
   Scrape cytology has been suggested as a noninvasive and reliable, rapid diagnostic screening method for mammary PD
   
  Punch, wedge, or excisional biopsy of the lesional skin of the nipple-areola complex to include the dermal and subcutaneous tissue for detailed microscopic examination provides an adequate sample for the accurate diagnosis of mammary PD.  
  
  
  Tumorous Paget cells are negative for estrogen and progesterone receptor sites,
   
   Mammary Paget disease has been classified into 4 clinical stages.
  

  • Stage 0 - Lesion confined to the epidermis, without underlying in situ ductal carcinoma of the breast
  • Stage 1 - Associated with in situ ductal carcinoma just beneath the nipple
  • Stage 2 - Associated with extensive in situ ductal carcinoma
  • Stage 3 - Associated with invasive ductal carcinoma
     
     Mastectomy (radical or modified) and lymph node clearance are appropriate therapies for patients with mammary Paget disease (PD) with a palpable mass and underlying invasive breast carcinoma. As many as two thirds of patients are reported to have axillary lymph nodes positive for metastasis. Noninvasive breast carcinoma (in situ carcinoma) is found in about 65% of patients with mammary PD without a palpable mass