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Year : 2010  |  Volume : 6  |  Issue : 1  |  Page : 95-96

Paget disease of the male nipple

Department of Surgery in National Institute of Oncology, Rabat, Morocco

Date of Web Publication15-May-2010

Correspondence Address:
T El Harroudi
BP 502, Oujda principal, Oujda
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.63561

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 > Abstract 

Breast cancer occurring in the mammary gland of men is infrequent. It accounts for 0.8% of all breast cancers, which is less than one per cent of all newly diagnosed male cancers and 0.2% of male cancer deaths. However, Paget disease of the male nipple is extremely rare. We report a single case of Paget disease with infiltrative ductal carcinoma of the breast in a 61-year-old man.

Keywords: Breast carcinoma, male nipple, Paget disease

How to cite this article:
El Harroudi T, Tijami F, El Otmany A, Jalil A. Paget disease of the male nipple. J Can Res Ther 2010;6:95-6

How to cite this URL:
El Harroudi T, Tijami F, El Otmany A, Jalil A. Paget disease of the male nipple. J Can Res Ther [serial online] 2010 [cited 2021 Sep 27];6:95-6. Available from: https://www.cancerjournal.net/text.asp?2010/6/1/95/63561

 > Introduction Top

Since the initial description in 1874, [1] mammary Paget disease has remained a rare disease that accounts for 1 to 5% of all breast cancers and may be associated with underlying breast malignant neoplasm. [2] It is uncommon in men. [3] We would like to report a single case of Paget disease of the male breast, which we observed in our hospital.

 > Case Report Top

A 61-year-old male was admitted for evaluation of a lesion on the left nipple. His nipple had become indurated, pruritic and with erythematous skin changes for the past year-and-a-half. Medical, family and social history records were unremarkable. Physical examination revealed a 3 cm, scaly, indurated plaque obscuring the normal architecture of the left nipple [Figure 1]. Lymphadenopathy was absent. He had no family history of breast cancer.

A mammogram showed a lesion highly suspicious of breast carcinoma [Figure 2]. The histopathologic diagnosis of biopsy of the lesion was infiltrative ductal carcinoma and Paget disease of the breast [Figure 3].

The patient underwent a left total mastectomy combined with axillary clearance of the nipple. The histology showed an invasive ductal carcinoma associated with Paget disease. The axillary nodes were free from metastases. Postoperative adjuvant therapy included chemo-radiotherapy. After two years, the patient continues to be disease-free.

 > Discussion Top

Paget disease of the breast was first described, in 1874, by Sir James Paget in 15 women who presented with eruptions of the areola and nipple associated with subsequent breast cancer. [1]

Breast cancer occurring in the mammary gland of men is infrequent, accounting for 0.8% of all breast cancers; less than 1% of all newly diagnosed male cancers and 0.2% of male cancer deaths. [4]

The mean age of men who present with Paget disease of the breast is 60 years, but ages range from 43 to 81 years. [2],[5] The average delay in treatment of the disease from the onset of symptoms is about eight months in men. Although men present earlier with symptoms and show no histologic differences from women their prognosis tends to be worse with a five-year survival rate of 20 to 30%. [5]

Since the histological appearance of the Paget disease of the breast was first described, its histogenesis has been surrounded by much controversy. Two main hypotheses have tried to explain its nature and origin: [6]

The first theory, the epidermotropic (ductal) theory, postulates that the Paget cells are, in origin, duct cancer cells that have migrated along the basal membrane of underlying ducts to the epidermis of the nipple. [7] This theory is supported by the presence of an underlying intraductal or invasive carcinoma in majority of the patients. [8]

The second theory, of in situ malignant transformation or degeneration from existing cells, regards the Paget cells as malignant keratinocytes appearing in situ and thus considers Paget disease of the breast to be an in situ carcinoma independent of any underlying carcinoma of the breast. [9] This theory is supported by histologic studies in cases with the absence of an underlying carcinoma of the breast. [9] Each of these theories is plausible; however, treatment approaches differ markedly depending on the theory of histogenesis. [6]

Paget disease of the breast clinically presents as nipple erythema and mild eczematous scaling and flaking, progressing to nipple crusting, skin erosions and ulceration. The condition spreads outward off the nipple and onto the areola and surrounding skin of the breast. [10] Temporary resolution of the eczematoid changes with or without the application of topical corticosteroids is possible and this may cause further delay in diagnosis. Paget disease of the breast may be asymptomatic and unsuspected clinically and may be reported as a histologic finding by the pathologist on a mastectomy specimen. [6] Approximately 50% of patients also present with an associated palpable mass in the breast. [6]

Paget disease is often mistaken for dermatitis or a benign dermatological condition involving the nipple. It is not unusual for the diagnosis to be delayed. A skin specimen containing Paget cells and a lactiferous duct secures the diagnosis and can be obtained by nipple scrape cytology or biopsy. More than 97% of patients with Paget disease have an underlying breast carcinoma. Paget may present with (54%) or without (46%) a mass. [10] Invasive breast cancer coexists with Paget disease in 93% of patients with a mass and in 38% of patients without a mass. Because of the relative scarcity of cases in men, no studies have objectively compared treatment techniques in this population. Treatment may include modified radical mastectomy, axillary staging, radiation therapy, or systemic chemotherapies. [3]

 > References Top

1.Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholome Hosp Rep 1874;10:87-9.  Back to cited text no. 1
2.O'Sullivan ST, McGreal GT, Lyons A, Burke L, Geoghegan JG, Brady MP. Paget disease of the breast in a man without underlying breast carcinoma. J Clin Pathol 1994;47:851-2.  Back to cited text no. 2
3.Bernardi M, Brown AS, Malone JC, Callen JP. Paget Disease in a Man. Arch Dermatol 2008;144:1660-2.  Back to cited text no. 3
4.Iglehart DJ, Kaelin CM. Diseases of the breast. In: Towsend CM, Beauchamp RD, Evers BM, Mattox KL, editors. Textbook of surgery.17 th ed. London: WB Saunders; 2004. p. 867-928.  Back to cited text no. 4
5.Serour F, Birkenfeld S, Amsterdam E, Treshchan O, Krispin M. Paget disease of the male breast. Cancer 1988;62:601-5.  Back to cited text no. 5
6.Sakorafas GH, Blanchard K, Sarr MG, Farley DR. Paget disease of the breast. Cancer Treat Rev 2001;27:9-18.  Back to cited text no. 6
7.Muir R. Pathogenesis of Paget disease of the nipple and associated lesions. Br J Surg 1935;22:728-37.  Back to cited text no. 7
8.Ashikari R, Park K, Huvos AG, Urban JA. Paget disease of the breast. Cancer 1970;26:680-5.  Back to cited text no. 8
9.Lagios MD, Westdahl PR, Rose MR, Concannon S. Paget disease of the nipple. Alternative management in cases without or with minimal extent of underlying breast carcinoma. Cancer 1984;54:545-51.  Back to cited text no. 9
10.Ucar AE, Korukluoglu B, Ergul E, Aydin R, Kusdemir A. Bilateral Paget's disease of the male nipple: First report. Breast 2008;17:317-8.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

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