炎性乳癌（附文献一则） / 开普饭

一般情况
l 女，45岁，已婚，末次月经2019-09-08
l 入院日期：2019-09-18
l 现病史：患者于6周前无意中发现右乳腺肿物，约鸡蛋样大小，伴胀痛及轻压痛，局部无红肿、发热，无胸闷，无咳嗽、咳痰，无恶心、呕吐。2019-08-08深圳市人民医院B超检查提示：双侧乳腺内多发性结节，性质待查，BIRA-DS 3类。近2周患者自觉胀痛明显加重，伴乳头下陷，遂来二院就诊。

炎性乳癌（IBC）
l 发病机制不清。
l 临床上常为急性炎症的表现，出现红、肿、热、痛，但也可没有此征。-本例有乳房胀、痛短期内明显加剧。
l 在组织学上无特殊性，乳腺癌的各种病理类型均可见于IBC。-本例浸润性导管癌III级
l 病理常见脉管癌栓。-本例阳性

l 主要与乳腺炎鉴别。主要鉴别点：
l       ①临床症状不如炎症明显，多无发热和白细胞升高，疼痛也不明显，皮肤改变较广泛，可见橘皮样改变及乳头凹陷；
l       ②MRI检查肿块信号有所不同：乳腺感染中发现的肿块多为脓肿，在T2WI呈典型高信号，有边缘强化；浸润性乳腺癌结缔组织增生反应中形成的纤维组织导致了T2WI低信号；
l       ③时间一信号曲线多呈流出型，而炎性病变时间一信号曲线多呈流入型；
l 影像学表现无特殊性，在诊疗过程中起辅助诊断作用
l 由于炎性乳癌是浸润性乳腺癌的一种非常恶性的形式，预后很差。尤其在诊断存疑时，特别是在短期严格抗炎治疗l周后临床症状未见减轻时，穿刺活检取病理诊断非常必要。

治疗
l 化疗及放疗
l 部分激素受体阳性者行内分泌治疗
l 是预后最差的乳腺癌类型
Breast ImagingFree Access
Paget Disease of the Breast: Mammographic, US, and MR Imaging Findings with Pathologic Correlation
Hyo Soon Lim ,
Su Jin Jeong,
Ji Shin Lee,
Min Ho Park,
Jin Woong Kim,
Sang Soo Shin,
Jin Gyoon Park,
Heoung Keun Kang
Author Affiliations
From the Departments of Radiology (H.S.L., S.J.J., J.W.K., S.S.S., J.G.P., H.K.K.), Pathology (J.S.L.), and Surgery (M.H.P.), Chonnam National University Medical School, 8 Hack Dong, Dong Ku, Gwangju 501-757, South Korea.
Address correspondence to H.S.L. (e-mail: nico1220@dreamwiz.com).
Published Online:Nov 8 2011https://doi.org/10.1148/rg.317115070
Abstract
Paget disease is a rare malignancy of the breast characterized by infiltration of the nipple epidermis by adenocarcinoma cells. The clinical features of Paget disease are characteristic and should increase the likelihood of the diagnosis being made. An important point is that more than 90% of cases of Paget disease are associated with an additional underlying breast malignancy. Paget disease is frequently associated with ductal carcinoma in situ (DCIS) in the underlying lactiferous ducts of the nipple-areolar complex; it may even be associated with DCIS or invasive breast cancer elsewhere in the breast, at least 2 cm from the nipple-areolar complex. Nevertheless, mammographic findings may be negative in up to 50% of cases. Magnetic resonance (MR) imaging can be useful in patients with Paget disease for evaluation of the nipple-areolar complex and identification of an additional underlying malignancy in the breast. The appropriate surgical treatment must be carefully selected and individualized on the basis of radiologic findings, especially those obtained with breast MR imaging.
© RSNA, 2011
LEARNING OBJECTIVES
After completing this journal-based CME activity, participants will be able to:
·.
Describe the clinical and pathologic features of Paget disease of the breast.
·.
Recognize the imaging findings of Paget disease of the breast at mammography, US, and MR imaging.
·.
Discuss the role of breast MR imaging in assessment of lesion extent and treatment planning.
Introduction
Paget disease of the breast is an uncommon disease, accounting for approximately 1%–3% of all cases of breast carcinoma (1,2). It was described by James Paget (3) in 1874 as a “syndrome in which ulceration of the nipple was invariably associated with an underlying cancer.” The prevalence of an associated cancer ranges from 67% to 100%, with most studies reporting the presence of a concurrent malignancy in over 90% of patients (1,4). Therefore, Paget disease of the nipple is nearly always a sign of underlying breast malignancy.
Underlying cancer is common even in women with a benign-appearing mammogram and no palpable mass. Patients with a palpable mass or suspicious mammographic findings have a high likelihood of invasive cancer, and these factors were significantly associated with a worse outcome (5). It is important to search for the associated radiologic abnormalities to detect the underlying malignancy and thus facilitate selection of the appropriate management. Breast magnetic resonance (MR) imaging is an effective diagnostic modality for detection of clinically and mammographically occult cancer; previous reports suggest the useful role of breast MR imaging in evaluation of patients with Paget disease (6–9).
In this article, we describe the histopathologic features, pathogenesis, and clinical manifestations of Paget disease of the breast; discuss the differential diagnosis of nipple changes and classification of Paget disease; and demonstrate the imaging findings of Paget disease at mammography, ultrasonography (US), and MR imaging. Management of Paget disease and the role of these imaging techniques in treatment planning are also discussed.
Histopathologic Features
Paget disease is characterized by infiltration of the epidermis of the nipple by malignant cells called Paget cells. A Paget cell is a large round or ovoid cell with pale, clear, abundant cytoplasm and an enlarged pleomorphic and hyperchromatic nucleus that invades the epidermis (Fig 1) (10). Paget cells may lie singly, primarily along the basal epidermal cells, or form small nests, sometimes as ductal or glandular structures.

Figure 1 Paget disease of the nipple. Photomicrograph (original magnification, ×100; hematoxylin-eosin [H-E] stain) shows a Paget cell (arrows), which is a large round cell with pale, clear, abundant cytoplasm and an enlarged pleomorphic nucleus that invades the epidermis.
The dermis shows reactive changes (plasma cell infiltration, formation of new capillaries, hyperemia, and serous exudation) (11). In this setting, there is invariably an underlying ductal carcinoma in situ (DCIS) with or without invasive ductal carcinoma. Among the histopathologic subtypes of intraductal carcinoma, Paget disease of the breast appears to be most commonly associated with the solid or comedo form (12,13).
Pathogenesis
There are two leading theories about the histogenesis of Paget disease. The first one is the epidermotropic theory. If invasive breast cancer or DCIS is already present in the breast, cells are thought to break away from the malignancy and migrate through the milk ducts along the basal membrane, where they enter the nipple and areola (14). This theory is supported by the presence of an underlying carcinoma in the majority of patients, as well as the fact that Paget cells and the underlying associated ductal carcinoma share the same immunohistochemical profile and patterns of gene expression (15,16).
The second theory is the intraepidermal transformation theory. In this theory, Paget cells arise in the epidermis of the nipple independently of the underlying carcinoma by means of in situ malignant transformation or degeneration of existing cells (4). This theory presumes that Paget disease of the breast is an independent process that has affected the epidermis of the nipple in the same way it has affected the underlying mammary parenchyma.
In a few cases of Paget disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. In those cases, nipple skin cells may change independently into cancer cells. This view is supported by the results of ultrastructural studies, which have demonstrated desmosomal attachments between Paget cells and adjacent keratinocytes in the epidermis. This finding goes against the migratory nature of the epidermotropic theory (17,18). This view is also supported by the presence of intermediate cells with characteristics of both keratinocytes and Paget cells (17).
Clinical Manifestations
Symptoms in patients with Paget disease include itching, eczema, erythema of the nipple and areola, nipple erosion or ulceration, scaly or flaky skin, nipple retraction, bloody discharge from the nipple, or a combination of these (Fig 2) (1). It is important to know the clinical manifestations of Paget disease because they can be the only signs of the breast cancer.
Temporary resolution of the eczematous changes with or without the application of topical corticosteroids is possible, and this may cause a further delay in diagnosis (19).
Figure 2 Clinical features of Paget disease in a 36-year-old woman with a 2-year history of pruritic erythema of the left nipple. She had a history of recurrent nipple eczema, which was treated at an outside clinic with topical ointment. A skin biopsy was performed, and Paget disease was confirmed. (a) Photograph shows an erythematous scaly patch with oozing and crusting in the areola that effaces the nipple. (b) Mediolateral oblique mammogram of the left breast shows periareolar skin thickening (arrowhead) and diffuse fine pleomorphic microcalcifications. The patient underwent a mastectomy, and invasive ductal carcinoma was diagnosed.

A physical examination should be performed to reveal a potential palpable mass or thickening of the parenchyma. Approximately 50% of patients with Paget disease also present with an associated palpable mass in the breast. Among patients presenting with a palpable mass, approximately 90%–94% are found to have invasive disease, whereas a lesion without a palpable mass is usually associated with DCIS (1,10,12,20).
Differential diagnosis from benign eczema is important. Benign eczema is usually bilateral, may be associated with systemic symptoms of atopic dermatitis, and responds to a topical steroid (21). If symptoms do not improve after treatment with a topical steroid, a biopsy should be performed. In cases of Paget disease, the delayed diagnosis resulting from treatment with topical steroids aggravates the underlying malignancy. In the series reported by Paget (3), all patients ultimately developed breast carcinoma within 1 year of the nipple changes (Fig 3).
Figure 3 Paget disease in a 55-year-old woman with a 1-year history of pruritic erythematous change of the left nipple. She had a history of recurrent nipple eczema, which was treated at an outside clinic. (a) Craniocaudal mammogram shows thickening of the nipple-areolar complex without a definite mass or microcalcifications in the breast. A skin biopsy was performed, and Paget disease was confirmed. The patient refused treatment for the disease. (b) Craniocaudal mammogram obtained 2 years later shows areolar skin thickening, nipple retraction, and diffuse, scattered, fine pleomorphic and linear microcalcifications in the entire breast including the subareolar region. The microcalcifications were not seen at mammography performed 2 years earlier.

Unlike the patients with findings suggestive of Paget disease of the nipple, there are patients with Paget disease but without evident clinical findings. Paget cells are found in the nipple at histopathologic examination, usually at mastectomy. They manifest as a palpable mass or nipple discharge or are detected during a screening examination. There are general trends in the relationship between the extent of cancer and the manner in which Paget disease is discovered. Patients first seen with clinical nipple changes had less extensive cancers in comparison with patients in whom Paget disease was clinically occult and found at mastectomy (13).
Differential Diagnosis of Nipple Changes
Both benign and malignant processes may produce visible changes in the skin of the nipple, including erythema, scaling, crusting, fissures, vesicles, erosions, lichenification, or some combination of these. Correct diagnosis of the underlying pathologic process is important because of the possibility of a breast malignancy. Histologic analysis of a biopsy specimen is often necessary for accurate diagnosis (22).
Benign processes that cause changes in the nipple-areolar complex are eczema, psoriasis, allergic contact dermatitis, irritant dermatitis, and lichen simplex chronicus. Malignant processes that cause changes in the nipple-areolar complex are Paget disease and squamous cell carcinoma in situ (Bowen disease). Bowen disease cannot be clinically differentiated from Paget disease, and a biopsy is necessary for diagnosis (22).
Classification
Paget disease of the breast can be divided into three categories (Fig 4) (8): (a) Paget disease of the nipple without DCIS (Fig 5); (b) Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the nipple-areolar complex (Fig 6); and (c) Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the nipple-areolar complex and associated DCIS or invasive breast cancer elsewhere in the breast, at least 2 cm from the nipple-areolar complex (Fig 7).
Figure 4 Drawings show categories of Paget disease of the breast (red areas). (a) Paget disease of the nipple without DCIS. (b) Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the nipple-areolar complex. (c) Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the nipple-areolar complex and associated DCIS or invasive breast cancer elsewhere in the breast (at least 2 cm from the nipple-areolar complex).

Figure 5 Paget disease without DCIS in a 68-year-old woman with eczema and skin pigmentation of the left nipple. A punch biopsy was performed, and Paget disease of the nipple was diagnosed. (a) Craniocaudal mammogram shows skin thickening in the periareolar area of the left breast. The breast is heterogeneously dense with no discernible abnormality. A mastectomy was performed. (b) Photomicrograph (original magnification, ×100; H-E stain) of the histopathologic specimen shows Paget disease of the nipple, with Paget cells (arrows) appearing singly or in clusters in the epidermis. There was no malignant mass at histopathologic evaluation of the mastectomy specimen. Paget disease of the nipple without DCIS or invasive cancer in the subareolar region or elsewhere in the breast was diagnosed.

Figure 6 Associated DCIS in a 35-year-old woman with Paget disease of the right nipple. Mastectomy was performed. Photomicrograph (original magnification, ×40; H-E stain) of the histopathologic specimen shows Paget disease of the nipple with associated DCIS (arrows) in the underlying lactiferous ducts of the nipple-areolar complex.

Figure 7 Paget disease with associated invasive breast cancer in a 57-year-old woman with a 1-year history of eczema of the right nipple-areolar complex. (a) Mediolateral oblique mammogram of the right breast shows an irregular, spiculated, equal-density mass (arrow) at the 6-o'clock position and thickening of the nipple-areolar complex (arrowhead). An enlarged lymph node is visible in the axillary tail. (b) US image shows the irregular hypoechoic mass with spiculated margins. (c) Axial contrast material–enhanced maximum intensity projection MR image shows thickening and abnormal enhancement of the nipple-areolar complex (arrowhead) and the irregular spiculated enhancing mass (arrow). Mastectomy was performed. The pathologic diagnosis was invasive ductal carcinoma, type not otherwise specified, and Paget disease of the nipple.

In a study of nine cases of Paget disease, Frei et al (8) found one case without DCIS, four cases with DCIS in the underlying lactiferous ducts, and four cases with DCIS or invasive cancer elsewhere in the breast. In a study of 10 cases, Kim et al (23) found one case of Paget disease without DCIS. In a study of 34 cases, Morrogh et al (24) found two cases of Paget disease without DCIS.
Paget disease of the nipple without DCIS may be explained by the in situ transformation theory of pathogenesis. Alternatively, it could represent a case in which the primary breast tumor is not large enough to be found with our imaging techniques. This view would be supported by the previously mentioned cases in which the parenchymal malignancy was found 1–2 years after Paget disease of the nipple was diagnosed.
Diagnosis
The clinical features of Paget disease are characteristic and should alert the clinician to the likelihood of this disease with an underlying carcinoma, which should be evaluated radiologically. The clinical and imaging findings are complementary and should be correlated to confirm or exclude a diagnosis of Paget disease. Exfoliative cytology with demonstration of Paget cells may be useful, but a negative result can occur. Surgical biopsy is the diagnostic standard.
When a patient presents with nipple-areolar changes, a full-thickness biopsy of the nipple and areola is important for establishing a diagnosis (11). After diagnosis, radiologic evaluation of the breast to detect an underlying malignancy should be performed.
Imaging evaluation of the breasts with at least mammography should preferably be performed before biopsy, with follow-up US and possibly MR imaging added in some cases even before performance of surgical biopsy.
Mammography
Complete mammographic evaluation should be performed in all cases with clinical findings of Paget disease to detect the underlying carcinoma, to exclude multifocal disease, and to act as a tool for follow-up in patients selected to receive conservative treatment.
Findings that may be seen at mammography include nipple, areolar, and subareolar abnormalities, including skin thickening, nipple retraction, malignant calcifications, or a mass at the level of the nipple-areolar complex (Fig 8). Mammographic findings in breast parenchyma include a discrete mass or masses that demonstrate suspicious features, asymmetry, architectural distortion, or malignant calcifications elsewhere in the breast (Figs 7, 9) (13,25).
Although abnormal mammographic findings involving the nipple-areolar complex are not pathognomic for Paget disease of the breast, these findings should be correlated with physical findings and the clinical history to exclude Paget disease.
Figure 8 Paget disease with associated DCIS in a 61-year-old woman with a 1-year history of a pruritic erythematous scaly patch of the left nipple-areolar complex. Punch biopsy was performed, and Paget disease of the nipple was diagnosed. Bilateral mediolateral oblique mammograms show marked areolar and skin thickening (arrowhead in b) and a retroareolar mass (arrow in b) in the left breast. Histologic evaluation showed DCIS (solid type) confined to the nipple-areolar complex and Paget disease of the nipple.

Figure 9 Paget disease with associated invasive breast cancer in a 54-year-old woman with a palpable mass in the right breast and eczematous change of the right nipple-areolar complex. (a) Mediolateral oblique mammogram shows segmentally distributed fine pleomorphic microcalcifications in the upper outer right breast. Punch biopsy and percutaneous core needle biopsy were performed, and Paget disease with invasive ductal carcinoma was diagnosed. (b, c) Axial contrast-enhanced fat-suppressed subtraction T1-weighted MR images show segmental, clumped, non-masslike enhancement (arrow in b) in the upper outer right breast and asymmetric nodular enhancement in the right nipple (arrowhead in c).

However, the mammographic appearance can be normal in 22%–50% of patients (Fig 10) (13,25–27).
Sawyer and Asbury (25) reported that five (29%) of 17 cases of Paget disease had normal mammographic findings, while Ceccherini et al (26) reported that 12 (44%) of 27 cases had normal mammographic findings. Ikeda et al (13) reported that 50% of cases with typical clinical features of Paget disease were negative at mammography. In addition, the extent of disease was underestimated with mammography in up to 43% of cases (13,27).
Figure 10 Paget disease with associated DCIS in a 60-year-old woman with itching of the left nipple and bloody discharge from the left nipple. A punch biopsy was performed, and Paget disease of the nipple was diagnosed. Bilateral craniocaudal mammograms of the right (a) and left (b) breasts show scattered fibroglandular densities, with no discernible abnormality in the left breast. A mastectomy was performed at the patient's request. The pathologic diagnosis was DCIS (comedo type) in the lactiferous ducts of the nipple-areolar complex and Paget disease of the nipple.

Mammography has limited value in demonstrating the extent of disease. Evaluation with additional breast imaging modalities such as US or MR imaging should be considered, especially in patients who opt for breast conservation surgery, since Paget disease of the nipple is most often associated with underlying DCIS and also associated with invasive ductal carcinoma.
Ultrasonography
US may be helpful and should be considered part of the initial evaluation, especially when results of mammography are negative. A lesion that is invisible at mammography may be imaged with breast US.
Findings that may be seen at US include a mass, microcalcifications, ductal ectasia, or morphologic changes of the nipple-areolar complex such as flattening, asymmetry, and thickening (Figs 11, 12) (23). In one study, US depicted 43 masses in 35 patients with Paget disease and showed parenchymal heterogeneity in one patient. The masses demonstrated lobulation or an irregular contour and no posterior acoustic shadowing (Fig 7) (28).
Figure 11 Paget disease of the left nipple in a 63-year-old woman. (a) Mediolateral oblique mammogram shows diffuse thickening of the left nipple-areolar complex. No associated mass or microcalcifications are visible. (b) US image shows morphologic change of the nipple-areolar complex with flattening and asymmetric thickening (arrows).

Figure 12 Paget disease with associated DCIS in a 67-year-old woman with a palpable mass associated with retraction of the left nipple. (a) Mediolateral oblique mammogram of the left breast shows diffuse fine pleomorphic and linear microcalcifications in the central breast extending to the subareolar area and nipple retraction. (b) US image shows an irregular, ill-defined, hypoechoic lesion that contains microcalcifications with ductal ectasia. US-guided core needle biopsy revealed DCIS. Mastectomy was performed. (c) Photomicrograph (original magnification, ×100; H-E stain) of the histopathologic specimen shows Paget cells forming a nest (arrow) in the epidermis of the nipple.

However, not all underlying cancers are detected at breast US. In one study, breast cancer was occult at both mammography and US in 13% of 52 patients with Paget disease (28).
MR Imaging
MR imaging can have a role in preoperative evaluation of patients with Paget disease in assessment of the nipple-areolar complex as well as underlying breast cancer, especially when results of mammography or US are negative. After mammography with negative results, MR imaging allows detection of otherwise occult disease, accurately demonstrates the extent of disease, and permits underlying cancer to be ruled out (24).
Findings that may be seen at MR imaging include abnormal nipple enhancement, thickening and enhancement of the nipple-areolar complex, an associated enhancing DCIS or invasive tumor, or a combination of these (Figs 7, 9). There are clear differences and asymmetries in the characteristics of the nipple-areolar complex between the normal and abnormal sides. At contrast-enhanced MR imaging, the degree of normal nipple enhancement varies and there is a bilaterally symmetric enhancement pattern: The enhancement may be absent, mild, or intense (Fig 13) (29). Intense enhancement is due to the presence of numerous vessels (30). In contrast to the normal side, asymmetric nodular, discoid, or irregular enhancement may be seen in the abnormal nipple or nipple-areolar complex (Fig 14) (29,31).
Figure 13 Normal nipple enhancement patterns at MR imaging. Axial contrast-enhanced fat-suppressed subtraction T1-weighted MR images show various degrees of bilateral symmetric enhancement of the nipples: no enhancement (top), mild enhancement (middle), and intense enhancement (bottom).

Figure 14 Abnormal enhancement patterns of the nipple or nipple-areolar complex at MR imaging. Axial contrast-enhanced fat-suppressed subtraction T1-weighted MR images show asymmetric nodular (top), discoid (middle), and irregular (bottom) enhancement of the nipple or nipple-areolar complex (arrowhead). These findings are considered abnormal.

For diagnosis and evaluation of tumors of the nipple and retroareolar tumors, MR imaging has higher sensitivity than mammography. It also shows nipple involvement even when it is clinically unsuspected (29).
It is important to evaluate the entire breast even if a subareolar mass is noted at physical examination or mammography. The underlying cancer may not be located immediately beneath the nipple-areolar complex but may be present at a distant site with no apparent anatomic connection. Moreover, the reported prevalences of multifocality and multicentricity in Paget disease are 41% and 34%, respectively (27). MR imaging can be useful in detecting distant, multifocal, or multicentric lesions when there is no clinical sign or suspicious mammographic finding (Fig 15).
Figure 15 Paget disease with associated DCIS and invasive breast cancer in a 35-year-old woman with a palpable mass in the right breast. She underwent US-guided core needle biopsy at an outside clinic, and invasive ductal carcinoma was diagnosed. (a) Craniocaudal mammogram shows segmentally distributed microcalcifications (arrows) in the upper outer right breast. (b–d) Axial contrast-enhanced fat-suppressed subtraction T1-weighted MR images show asymmetric nodular enhancement in the right nipple (b) and segmental clumped, non-masslike enhancement in the upper outer right breast (arrowheads in c); the latter finding corresponds to the area of microcalcifications seen at mammography. In addition, regional non-masslike enhancement is seen in the upper inner right breast (arrow in c), and an oval mass with homogeneous early enhancement is seen in the upper central right breast (arrow in d). At physical examination, there was erythematous change of the right nipple. A punch biopsy revealed Paget disease of the nipple. A mastectomy was performed. The pathologic diagnosis was invasive ductal carcinoma, type not otherwise specified, and multicentric DCIS.

In patients without clinical evidence of Paget disease of the nipple—for instance, patients undergoing a screening examination or breast MR imaging for some other indication—identification of abnormal enhancement of the nipple or nipple-areolar complex at breast MR imaging should raise suspicions for Paget disease. Further, a careful clinical and physical examination should be performed.
However, asymmetric abnormal enhancement of the nipple or nipple-areolar complex is not pathognomic for Paget disease, since this finding can be seen in other entities such as nipple adenoma or extension of breast neoplasms toward the nipple (32,33). False-negative results of MR imaging peformed for evaluation of the nipple-areolar complex in cases of Paget disease have also been reported (Fig 16) (34).
Figure 16 Paget disease with associated invasive breast cancer in a 56-year-old woman with a palpable mass in the left breast. (a) US image shows an approximately 3-cm, irregular, indistinctly marginated, hypoechoic mass with microcalcifications. US-guided percutaneous core needle biopsy revealed invasive ductal carcinoma. (b) Axial contrast-enhanced fat-suppressed subtraction MR image shows the 3-cm irregular mass with heterogeneous early enhancement (arrow) and surrounding non-masslike enhancement in the central left breast. (c) Axial contrast-enhanced fat-suppressed subtraction MR image shows smooth thin rim enhancement of the left nipple (arrowhead). This finding was considered to represent normal enhancement. A mastectomy was performed. (d) Photomicrograph (original magnification, ×100; H-E stain) of the histopathologic specimen shows Paget cells involving the epidermis of the nipple (arrows).

Additional evaluation with MR imaging can help identify the underlying malignancy in patients with Paget disease in whom the cancer is occult at clinical examination or mammography. This capability ultimately assists in selecting patients for breast conservation surgery.
MR imaging–guided breast biopsy before surgical treatment can potentially resolve false-positive MR imaging findings, allowing conservative treatment to be planned.
Management
Mastectomy, with or without axillary dissection, has been considered the standard treatment in patients with Paget disease for many years. More recently, breast conservation therapy, which involves complete resection of the nipple-areolar complex followed by definitive radiation therapy, has been shown to be a viable alternative to mastectomy in treatment of Paget disease, since an estimated two-thirds of patients with Paget disease have an underlying cancer that is confined to the central quadrant of the breast (27,35,36).
Dalberg et al (37) reported similar survival rates for selected patients with Paget disease of the nipple treated with breast conservation surgery and for those treated with mastectomy. The size of the underlying tumor and lymph node status were the only independent prognostic factors for survival; mastectomy was not associated with better survival outcomes than those associated with central lumpectomy (38).
However, for Paget disease of the nipple associated with DCIS or invasive breast cancer elsewhere in the breast (at least 2 cm from the nipple-areolar complex), treatment should include complete resection of the underlying disease combined with excision of the nipple-areolar complex, followed by radiation therapy of the remaining breast tissue (36).
If conservative treatment is selected, the patient should be followed up carefully. Breast conservation surgery can be the treatment modality for a select group of patients with Paget disease. Breast MR imaging should be performed to evaluate the nature and extent of the underlying cancer when breast conservation treatment is considered for Paget disease of the breast.
Conclusions
The clinical features of Paget disease are relatively characteristic, and the clinician should be aware of the approximately 90% chance of a concurrent malignancy. Although mammography may depict changes in the nipple-areolar complex, a mass, or calcifications in the breast, mammographic findings in some patients are normal. MR imaging may have a role in preoperative evaluation of patients with Paget disease, especially when results of mammography or US are negative. MR imaging can be useful for assessment of the nipple-areolar complex as well as the underlying malignancy and for selection of patients for breast conservation treatment.
Presented as an education exhibit at the 2010 RSNA Annual Meeting.
For this journal-based CME activity, the authors, editor, and reviewers have no relevant relationships to disclose.
Abbreviations:
DCIS
ductal carcinoma in situ
H-E
hematoxylin-eosin
Article History
Received: Apr 4 2011
Revision requested: June 22 2011
Revision received: July 21 2011
Accepted: Aug 1 2011
Published online: Nov 8 2011
Published in print: Nov 2011