Paget's disease of the breast overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]
Overview
Paget's disease is an eczematous skin change of the nipple that is usually associated with an underlying breast malignancy. Paget's disease of the breast was first discovered by James Paget, a British surgeon and physiologist, in 1874.[1] Paget's disease of the breast occurs in 1 – 4% of all female breast carcinoma cases and is invariably associated with underlying malignancy. On gross pathology, eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration of the nipple, and inversion of the nipple are characteristic findings of Paget's disease of the breast.[1] On microscopic histopathological analysis, epidermal Paget cells which are malignant glandular epithelial cells organized in groups with nest-like patterns or gland-like structures and are preferably located in the epidermal basal layer are characteristic findings of Paget's disease of the breast. Common risk factors in the development of Paget's disease of the breast are age, personal history of breast cancer, family history of breast cancer, irradiation, and genetic mutations. Symptoms of Paget's disease of the breast include itching, redness, thickened skin, and ulceration of the nipple. Common physical examination findings of Paget's disease of the breast include eczematous appearance of the nipple associated with yellowish or bloody discharge. Paget's disease of the breast must be differentiated from atopic dermatitis, eczema, psoriasis, malignant melanoma, Bowen's disease, basal cell carcinoma, and pagetoid dyskeratosis.[1][2] Biopsy may be helpful in the diagnosis of Paget's disease of the breast. Mammogram may be performed to detect the breast cancer associated with Paget's disease of the breast. Mastectomy is the mainstay of treatment for Paget's disease of the breast. Adjunctive radiation may be required.
Historical Perspective
Paget's disease of the breast was first discovered by James Paget, a British surgeon and physiologist, in 1874.The characteristic erythema and eczematous changes of the nipple seen with Paget’s disease of the breast were first described by Velpeau in 1856. The correlation between intraductal cancer and Paget’s disease of the breast was by Jacobeus in 1904.The background for the epidermotropic theory, that ducts containing carcinoma cells were apparently connected to overlying nipples containing Paget’s cells, was demonstrated by Muir and Inglis in 1939 and 1946 respectively. The first case of Paget's disease in a male was described by Elbogen in 1908.
Classification
There is no classification system established for Paget's disease of the breast.
Pathophysiology
On gross pathology, eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration of the nipple, and inversion of the nipple are characteristic findings of Paget's disease of the breast.[1] On microscopic histopathological analysis, epidermal Paget cells which are malignant glandular epithelial cells organized in groups with nest-like patterns or gland-like structures and are preferably located in the epidermal basal layer are characteristic findings of Paget's disease of the breast.
Causes
The cause of Paget's disease of the breast has not been identified.
Differential Diagnosis
Paget's disease of the breast must be differentiated from atopic dermatitis, eczema, psoriasis, malignant melanoma, Bowen's disease, basal cell carcinoma, and pagetoid dyskeratosis.[1][2]
Epidemiology and Demographics
Risk Factors
Common risk factors in the development of Paget's disease of the breast are age, personal history of breast cancer, family history of breast cancer, irradiation, and genetic mutations.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Paget's disease of the breast.[3]
Natural History, Complications and Prognosis
Diagnosis
Staging
There is no established system for the staging of Paget's disease of the breast.
History and Symptoms
Symptoms of Paget's disease of the breast include itching, redness, thickened skin, and ulceration of the nipple.
Physical Examination
Common physical examination findings of Paget's disease of the breast include eczematous appearance of the nipple associated with yellowish or bloody discharge.
Biopsy
Biopsy may be helpful in the diagnosis of Paget's disease of the breast. Findings on biopsy diagnostic of Paget's disease of the breast include presence of Paget cells, which are arranged in solid groups.[1]
Ultrasonography
Ultrasonography of the breast may be performed to detect the breast cancer associated with Paget's disease of the breast.
MRI
MRI may be performed to detect the breast cancer associated with Paget's disease of the breast.
Other Imaging Findings
Mammogram may be performed to detect the breast cancer associated with Paget's disease of the breast.
Other Diagnostic Studies
No additional tests are recommended for the diagnosis of Paget's disease of the breast.
Treatment
Medical Therapy
The predominant therapy for Paget's disease of the breast is surgical resection. Adjunctive radiation may be required.
Surgery
Mastectomy is the mainstay of treatment for Paget's disease of the breast. Patients who do not have a palpable lump are treated with removal of the nipple and areola, followed by whole-breast radiation therapy, whereas patients with associated ductal carcinoma in situ or invasive breast cancer are treated with complete resection of the underlying disease with excision of the nipple-areola complex and radiation therapy of the remaining breast tissue. When lymph nodes are involved, more extensive axillary lymph node surgery may be needed.
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Lopes Filho, Lauro Lourival; Lopes, Ione Maria Ribeiro Soares; Lopes, Lauro Rodolpho Soares; Enokihara, Milvia M. S. S.; Michalany, Alexandre Osores; Matsunaga, Nobuo (2015). "Mammary and extramammary Paget's disease". Anais Brasileiros de Dermatologia. 90 (2): 225–231. doi:10.1590/abd1806-4841.20153189. ISSN 1806-4841.
- ↑ 2.0 2.1 Gaspari, Eleonora; Ricci, Aurora; Liberto, Valeria; Scarano, Angela Lia; Fornari, Maria; Simonetti, Giovanni (2013). "An Unusual Case of Mammary Paget's Disease Diagnosed Using Dynamic Contrast-Enhanced MRI". Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/206235. ISSN 2090-6862.
- ↑ Paget's disease of the breast. U.S. Preventive Services Task Force.http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=breast+cancer