Paget Disease of the Breast

Myah S Ramirez, MD
The Operative Review of Surgery. 2023; 1:17-19

Download PDF

Table of Contents

General Information
Diagnosis & Treatment

References

General Information

Definition

  • A Rare Type of Breast Cancer with Paget Cells Involving the Nipple-Areola Complex
  • Also Known as Paget’s Disease of the Nipple or Mammary Paget Disease

Pathophysiology

  • Epidermotropic Theory: Paget’s Disease Derives from Underlying Breast Cancer with Malignant Ductal Cells Migrating Through the Ductal System to the Nipple 2
    • *By Far the More Commonly Accepted Theory for the Majority of Cases
  • Transformation Theory: Paget’s Disease Derives from Malignant Transformation of Nipple-Areolar Keratinocytes Independent of Underlying Disease 2
  • Dual Origin Theory: Depending on Circumstances, Paget’s Disease May Originate from Either Ductal Cancer Cells or Malignant Transformation of Keratinocytes 2

Underlying Malignancy

  • Paget’s Indicates an Underlying Ductal Carcinoma In Situ (DCIS) or Invasive Ductal Carcinoma (IDC) in 80-90% of Cases 3
  • 1-4% of All Breast Cancer Patients Also Have Paget’s Disease of the Breast 4
  • About 50% of Cases Have are Associated with a Palpable Mass 5
    • Mass is Most Common Centrally Located but Can Be Seen in Periphery with Multifocal or Multicentric Disease 5
  • May Be Seen as a Form of Local Recurrence After Nipple-Sparing Mastectomy 6

Epidemiology

  • Most Common in Postmenopausal Women Aged 50-60 5
  • Reported Age Range: 20-90 Years 7
  • Majority in Women (97.9%) But Can Affect Men (2.1%) 7

Presentation 3,8

  • Eczematoid Scaling of the Nipple-Areola Complex
  • Pain, Itching, & Burning
  • May Have Vesicles or Ulcerations
  • Copious Clear-Yellow Exudate – Later Feature 9
  • Flattened or Inverted Nipple – Later Feature 9
  • *Often Misdiagnosed as Eczema, Psoriasis, or Dermatitis 3,10

Paget Disease of the Breast 1

Diagnosis & Treatment

Diagnosis

  • Should Undergo a Bilateral Diagnostic Mammogram to Evaluate Underlying Lesion 8,9
    • May Also Consider an MRI to Evaluate for More Occult Lesions that May Be Missed on Mammogram 11
  • Definitive Diagnosis: Full Thickness (Punch) Skin Biopsy of the Nipple 9
    • May Also require Core Needle Biopsy (CNB) to Diagnose the Primary Lesion
  • Pathology: Paget Cells are Malignant Adenocarcinoma Cells within the Epidermis of the Nipple
    • Large, Pale-Staining Cells with Round or Oval Nuclei & Large Nucleoli Located Between the Normal Keratinocytes 12
    • Does Not Invade Through the Dermal Basement Membrane 12

Staging

  • Classified as Carcinoma In Situ (Tis) 13
  • Paget’s Disease of the Breast Does Not Change the Stage of an Underlying Malignancy 13

Treatment

  • General Treatment: Simple Mastectomy & Sentinel Lymph Node Biopsy (SLNB)
    • All Patients Require Resection of the Nipple-Areola Complex 9
  • May Consider Breast-Conserving Treatment (BCT) in Select Cases 14
    • Includes: Wide Local Excision (WLE) of the Nipple, Lumpectomy, SNLB, and Whole Breast Radiation
    • Consider Preoperative Evaluation with MRI to Rule Out Occult Multicentric Disease 15
    • Exact Indications for BCT are Not Yet Well Defined
  • 5-Year Survival Rate After Surgery: 82.5% 7

References

  1. Chu L. Wikimedia Commons. (License: Public Domain)
  2. Lim HS, Jeong SJ, Lee JS, Park MH, Kim JW, Shin SS, Park JG, Kang HK. Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics. 2011; 31(7):1973–1987.
  3. American Cancer Society. Paget Disease of the Breast. 2021. https://www.cancer.org/cancer/breast-cancer/about/types-of-breast-cancer/paget-disease-of-the-nipple.html
  4. Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, Yepes MM, Lippman ME. Paget’s disease of the nipple. Breast Cancer Res Treat. 2013; 141,1–12.
  5. Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, Yepes MM, Lippman ME. Paget’s disease of the nipple. Breast Cancer Res Treat. 2013 Aug;141(1):1-12.
  6. Lohsiriwat V, Martella S, Rietjens M, Botteri E, Rotmensz N, Mastropasqua MG, Garusi C, De Lorenzi F, Manconi A, Sommario M, Barbieri B, Cassilha M, Minotti I, Petit JY. Paget’s disease as a local recurrence after nipple-sparing mastectomy: clinical presentation, treatment, outcome, and risk factor analysis. Ann Surg Oncol. 2012 Jun;19(6):1850-5.
  7. Sisti A, Huayllani MT, Restrepo DJ, Boczar D, Advani P, Lu X, Spaulding AC, Ball CT, McLaughlin SA, Forte AJ. Paget disease of the breast: A national retrospective analysis of the US population. Breast Dis. 2020;39(3-4):119-126. 
  8. National Health Service. Paget’s Disease of the Nipple. 2019. https://www.nhs.uk/conditions/pagets-disease-nipple/#:~:text=Paget’s%20disease%20of%20the%20nipple%2C%20also%20known%20as%20Paget’s%20disease,the%20tissue%20behind%20the%20nipple.
  9. Miller ME, Morrow M. Uncommon Presentation of Cancer Affecting the Breast. In: Dixon JM, Barber MD. eds. Breast Surgery: A Companion to Specialist Surgical Practice, 6e. Elsevier. 2019.
  10. National Cancer Institute. Paget Disease of the Breast. 2012. https://www.cancer.gov/types/breast/paget-breast-fact-sheet
  11. Chen CY, Sun LM, Anderson BO. Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer. 2006 Oct 01;107(7):1448-58.
  12. Klimberg VS, Hunt KK. Diseases of the Breast. In: Townsend CM. eds. Sabiston Textbook of Surgery, 21e. Elsevier. 2021.
  13. National Cancer Institute. TNM Data: Breast Cancer: Pathologic T Staging. https://staging.seer.cancer.gov/tnm/input/1.9/breast/path_t/?breadcrumbs=(~schema_list~),(~view_schema~,~breast~)
  14. Hudson-Phillips S, Cox K, Patel P, Al Sarakbi W. Paget’s disease of the breast: diagnosis and management. Br J Hosp Med (Lond). 2023 Jan 2;84(1):1-8.
  15. Helme S, Harvey K, Agrawal A. Breast-conserving surgery in patients with Paget’s disease. Br J Surg. 2015 Sep;102(10):1167-74.