Paget’s Disease of the Breast

Published on 31/05/2015 by admin

Filed under Obstetrics & Gynecology

Last modified 22/04/2025

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Chapter 159 Paget’s Disease of the Breast

INTRODUCTION

Description: Paget’s disease of the breast is a malignant process that involves the nipple and areola. Rarely, it may also involve the skin of the vulva.
Prevalence: Two percent of breast cancers.
Predominant Age: Menopausal and perimenopausal.
Genetics: No genetic pattern.

ETIOLOGY AND PATHOGENESIS

Causes: Thought to arise in the dermoepidermal junction from multipotent cells that can differentiate into either glandular or squamous cells.
Risk Factors: None known.

CLINICAL CHARACTERISTICS

Signs and Symptoms

• Pruritic, red, eczematoid skin lesion, often associated with bleeding and crusting
• Almost always associated with infiltrating or intraductal carcinoma in deeper parts of the breast

DIAGNOSTIC APPROACH

Differential Diagnosis

• Eczema of the nipple
• Inflammatory breast cancer
• Chronic nipple irritation (jogger’s nipples)

Associated Conditions: Infiltrating or intraductal carcinoma.

Workup and Evaluation

Laboratory: No evaluation indicated.
Imaging: Mammography to detect deeper lesions and lesions in the contralateral breast.
Special Tests: A touch smear obtained by softening the crust with saline and gently scraping the surface often demonstrates the characteristic Paget’s cells.
Diagnostic Procedures: History, physical examination, and biopsy.

Pathologic Findings

Dermal infiltrates of large neoplastic cells (Paget’s cells). These cells have abundant clear cytoplasm with mucin and irregular prominent nucleoli. Most often, these cells arise from infiltrating ductal carcinoma.

MANAGEMENT AND THERAPY

Nonpharmacologic

General Measures: Evaluation, mammography.
Specific Measures: Therapy is focused on treatment of the underlying malignancy. When limited to the nipple, breast conservation may be possible.
Diet: No specific dietary changes indicated.
Activity: No restriction.
Patient Education: Instruction on monthly breast self-examination; American College of Obstetricians and Gynecologists Patient Education Pamphlet AP026 (Detecting and Treating Breast Problems), AP076 (Mammography), AP145 (Breast Self-Examination).

Drug(s) of Choice

None. Adjunctive chemotherapy is often recommended based on cell type and stage.

FOLLOW-UP

Patient Monitoring: Increased surveillance for recurrence or the development of tumors in the contralateral breast.
Prevention/Avoidance: None.
Possible Complications: Progression and spread of the underlying malignancy. Local skin erosion with bleeding and discharge.
Expected Outcome: Local recurrence is common.

MISCELLANEOUS

Pregnancy Considerations: Generally not a consideration. No direct effect on pregnancy.
ICD-9-CM Codes: Based on location and severity of disease.

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REFERENCES

Level I

Bijker N, Rutgers EJ, Duchateau L, et alEORTC Breast Cancer Cooperative Group. Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 2001;91:472.

Level II

Davis AB, Patchefsky AS. Basal cell carcinoma of the nipple: case report and review of the literature. Cancer. 1977;40:1780.

Marshall JK, Griffith KA, Haffty BG, et al. Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer. 2003;97:2142.

Level III

Dixon JM, Sainsbury JR, Rodger A. ABC of breast diseases. Breast cancer: treatment of elderly patients and uncommon conditions. BMJ. 1994;309:1292.

Jamali FR, Ricci AJr, Deckers PJ. Paget’s disease of the nipple-areola complex. Surg Clin North Am. 1996;76:365.

Lloyd J, Flanagan AM. Mammary and extramammary Paget’s disease. J Clin Pathol. 2000;53:742.

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