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Paget Disease of the Breast

Shannon A. Sanchez, MS, MD and Ariana M. Rosado Maldonado, M.D. Reviewed 04/2024
 


BASICS

DESCRIPTION

  • Rare cutaneous malignancy of the nipple-areola complex (NAC) characterized by epidermal adenocarcinoma cells, called Paget Cells, typically associated with underlying in situ or inva...

DIAGNOSIS

HISTORY

  • Scaly, raw, vesicular, or ulcerated lesion that begins on the nipple and then spreads to the areola

  • Pain, burning, and pruritus may be present even before clinically evident disease.

  • Ni...

TREATMENT

MEDICATION

  • Systemic adjuvant chemotherapy and/or endocrine therapy is based on the stage and hormone receptor status of the associated cancer.

  • In Paget disease without an associated cancer or ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Oncology consultation 

Patient Monitoring

  • After successful treatment for Paget disease of the breast, patients should start an individualized cancer surveillance pr...

REFERENCES

1
Sisti A, Huayllani MT, Restrepo DJ, et al. Paget disease of the breast: a national retrospective analysis of the US population. Breast Dis. 2020;39:119-126.
2
Markarian S, Holmes DR. Mammary...

ADDITIONAL READING

National Cancer Institute. Paget disease of the breast.  http://www.cancer.gov/types/breast/paget-breast-fact-sheet. Accessed July 15, 2022.  

CODES

ICD10

  • C50.019 Malignant neoplasm of nipple and areola, unsp female breast

  • C50.011 Malignant neoplasm of nipple and areola, right female breast

  • C50.012 Malignant neoplasm of nipple and areola, left ...

CLINICAL PEARLS

  • Any chronic or nonhealing nipple or breast lesions should be biopsied to rule out malignancy.

  • Further imaging is indicated to rule out additional underlying breast malignancy.

  • Breast mam...

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