© 2005 Foundation for Promotion of Cancer Research
Case Report |
MRI Accurately Depicts Underlying DCIS in a Patient with Paget's Disease of the Breast Without Palpable Mass and Mammography Findings
1 Department of Surgery and 2 Department of Pathology, Sakata Municipal Hospital, Sakata, Yamagata and 3 Department of Surgical Oncology, Tohoku University School of Medicine, Sendai, Japan
For reprints and all correspondence: Goro Amano, Department of Surgery, Sakata Municipal Hospital, 2-3-20, Sengoku-cho, Sakata 998-8585, Japan. E-mail: gamano{at}hospital.sakata.yamagata.jp
Received July 29, 2004; accepted November 2, 2004
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Abstract |
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 TOP Abstract INTRODUCTIONCASE REPORTDISCUSSIONReferences |
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Breast-conserving therapy must be carefully indicated among patients with Paget's disease of the breast, because the disease is often associated with an underlying in situ or invasive carcinoma, even when there are no palpable mass or mammography findings. We report a 52-year-old woman who complained of skin color change of her right nipple for 11 months. No mass was palpable in her breasts, and mammography did not show any density or calcification. Nipple biopsy revealed Paget's disease of the breast with ductal carcinoma in situ (DCIS) in the breast epithelium just beneath the nipple. Magnetic resonance imaging (MRI) of the breast demonstrated diffuse segmental enhancement in two different quadrants. According to the pattern of enhancement, the lesions depicted by MRI were diagnosed as an extensively spreading type of DCIS. Based on informed consent, the patient received a total mastectomy. The histopathological examination demonstrated non-invasive ductal carcinoma with comedo-necrosis. The histological mapping with subserial sectioning demonstrated an extent of the lesions that corresponded accurately to the lesions defined by MRI. We conclude that MRI may play an important role in selecting candidates for breast-conserving therapy out of those patients with mammary Paget's disease with no clinical evidence of an underlying breast carcinoma.
Key Words: Paget's disease • breast carcinoma • MRI • ductal carcinoma in situ
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INTRODUCTION |
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TOPAbstractINTRODUCTIONCASE REPORTDISCUSSIONReferences |
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The treatment for patients with Paget's disease of the breast is controversial. The standard treatment has been mastectomy (1,2). However, some studies have proposed the use of breast-conserving therapy for patients with Paget's disease in whom an underlying breast cancer cannot be located (3,4).
Nevertheless, other investigators reported that wide local excision of the nipple–areola complex and underlying breast tissue (cone excision) would have been insufficient surgery in 40% of their cases with no palpable mass and a normal mammogram, because of the multicentricity of the disease (5). Therefore, candidates for breast-conserving therapy must be selected carefully on an individual basis among those patients who have Paget's disease of the breast (6–8).
Here we report a case of a mammary Paget's disease patient who did not present either palpable mass or mammography findings. Magnetic resonance imaging (MRI) of the breast was very useful for making a decision on the appropriate surgical procedure.
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CASE REPORT |
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TOPAbstractINTRODUCTIONCASE REPORTDISCUSSIONReferences |
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A 52-year-old woman visited our hospital in January 2004 for an 11 month history of skin color change of her right nipple. She had also worried about the exudates from the nipple for 3 months. Physical examination showed the flattening and scaling of the nipple (Fig. 1). No mass was palpable in her breasts, and mammography did not reveal any density or calcification. There was also no abnormal finding on ultrasonography. Exfoliative cytology of the nipple demonstrated Paget's cells. To determine the surgical procedure of choice, MRI of the right breast was investigated. There were diffuse segmental enhancements existing in the upper and lateral quadrants (Fig. 2). According to the pattern of enhancement, the lesions depicted by MRI were diagnosed as an extensively spreading type of ductal carcinoma in situ (DCIS). In an attempt to excise all the suspicious lesions, we recommended mastectomy to the patient, to which she gave her consent.
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Prior to mastectomy, nipple biopsy was performed to confirm the histopathlolgical diagnosis of Paget's disease (Fig. 3A). Additional DCIS was also demonstrated in the breast epithelium just beneath the nipple (Fig. 3B).
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On March 1, 2004, modified radical mastectomy was performed. The specimen was subserially sectioned in 7 mm thick slices, and every block was examined histopathologically. Non-invasive ductal carcinoma with comedo-necrosis was present (Fig. 4). Cytonuclear grade was grade 2 and estrogen/progesterone receptor status was negative. HER-2 was strongly (3+) immunoreactive. These cytological and immunohistochemical results were similar to those of Paget's cells in the nipple biopsy specimen. Lymph node metastasis was not detected. According to the cancer map, DCIS were demonstrated extensively in the upper and lateral quadrants (Figs 5 and 6), accurately corresponding to the lesions shown by MRI (Fig. 2).
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DISCUSSION |
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TOPAbstractINTRODUCTIONCASE REPORTDISCUSSIONReferences |
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Paget's disease of the breast is a rare malignancy of the nipple–areola complex, comprising 0.5–5% of all breast cancer (1,7,9). It is manifested by progressive eczematoid changes of the areola with persistent soreness or itching (1,2,7). There have been debates about the histogenesis of this disease. According to the fact that this disease has been reported to be associated with an underlying breast carcinoma in 87–100% of cases (1–3,5–10), the epidermotropic theory, which postulates that Paget's cells are ductal cells that have migrated from an underlying breast carcinoma to the epidermis of the nipple, seems acceptable. The present case, where there existed underlying DCIS spreading extensively, is also compatible with the epidermotropic theory.
The treatment for patients with Paget's disease of the breast is controversial. Those patients with a palpable mass have a much greater incidence of invasive cancer, multifocal diseases, lymph nodal involvement and poor prognosis (1,2,6–8,10). Therefore, modified radical mastectomy is often the most appropriate treatment for patients with Paget's disease with a palpable lesion. On the other hand, for patients with Paget's disease who present no palpable mass, some investigators have proposed the use of breast-conserving therapy. Bijker et al. demonstrated that cone excision and radiotherapy is a feasible alternative for patients with Paget's disease and a limited extent of underlying DCIS (3). They reported a 5-year local recurrence rate of 5.2%. Marshall et al. also recommended local excision and definitive breast irradiation as an alternative to mastectomy for patients with Paget's disease presenting no palpable mass or mammographic density (4). In their report, 5- and 10-year local control rates are 91 and 83%, respectively.
On the contrary, Kothari et al. warned against using breast-conserving therapy (5). They retrospectively reviewed the cases of 70 women with a clinical diagnosis of Paget's disease. Despite the fact that only one-third of women presented with a palpable mass, the malignancy was frequently extensive, being confined to the retroareolar region in only 25% of cases. They also demonstrated that the true extent of the disease was underestimated by mammography in 43% of cases. Of the 10 patients with no palpable mass and a normal mammogram, 40% had multicentric or multifocal carcinoma which would have been incompletely excised by cone excision of the nipple. Fu et al. described that of eight patients with no palpable mass who had been treated by quadrantectomy, two (25%) patients had recurrence (8). They concluded that even if the patient has no palpable mass, conservative surgery should be selected cautiously because of a higher recurrence rate and multifocal lesions.
In an era when breast-conserving surgery is sometimes recommended even for advanced infiltrating breast tumors, it seems quite reasonable to propose breast-conserving therapy for patients with Paget's disease with no definitive underlying breast cancer. However, as is already widely known, Paget's disease of the breast has a very high incidence of being accompanied by an underlying invasive or in situ carcinoma, even when there is no palpable mass (1–6,8–10). Mammography often fails to demonstrate the true extent of the disease (3–6,9). A more accurate and reliable imaging modality is necessary to select candidates for breast-conserving therapy more safely from among the patients who have Paget's disease of the breast.
Clinical utilization of MRI for breast cancer diagnosis has been under investigation since the late 1970s. With advances in surface coil technology and new imaging protocols using intravenously administered gadopentetate dimeglumine, MRI of the breast can now detect invasive cancer with 98–100% sensitivity (11,12). Amano et al. demonstrated that MRI can also detect the extensively spreading type of DCIS, that is often occult clinically and mammographically, as a pattern of diffuse segmental enhancement (13). In their study, the sensitivity of MRI to detect the extensively spreading type of DCIS was calculated to be up to 100%, and the specificity was estimated as high as 95%. The role of MRI in determining the extent of breast cancer is now well established (14). In the present case, no mass was palpable in her breasts, neither was there any abnormal findings on mammography or ultrasonography. There seemed a chance for her right breast to be treated conservatively, and for that reason we investigated it by MRI. There were diffuse segmental enhancements in the upper and lateral quadrants, strongly indicating the extensively spreading type of DCIS. Post-operative histopathological examination demonstrated non-invasive ductal carcinoma with comedo-necrosis in the upper and lateral quadrants. The histological mapping with subserial sectioning demonstrated an extent of the lesions that corresponded accurately to the lesions defined by MRI. We conclude that MRI may play an important role in selecting candidates for breast conserving therapy out of those patients with mammary Paget's disease with no clinical evidence of an underlying breast carcinoma.
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References |
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