- Pictorial Review
- Open Access
Spectrum of Imaging Findings in Paget’s Disease of the Breast—A Pictorial Review
© The Author(s) 2015
- Received: 23 April 2015
- Accepted: 12 June 2015
- Published: 5 July 2015
We aimed to demonstrate imaging features of Paget’s disease of breast, which is an extremely uncommon malignancy that presents with changes in the nipple-areolar region that may or may not be associated with an underlying in situ component or invasive cancer.
Methods and Results
Mammography is the initial investigation of choice, having a high sensitivity especially in cases where a palpable mass is present. The addition of ultrasound improves the accuracy of mammography. When both mammography and ultrasound are negative, MRI may detect an underlying mass or ductal carcinoma in situ (DCIS).
The surgical management of Paget’s disease includes mastectomy with or without axillary dissection, though breast conservation surgery in the form of wide local excision can also be done in a selected group of patients. Management should be based on both clinical and imaging findings, including mammography and ultrasound, with MRI playing a crucial role in defining the extent of involvement.
• To differentiate Paget’s disease from other chronic skin conditions.
• Mammographic and ultrasound findings of histopathologically established Paget’s disease.
• When ultrasound and mammogram are negative, MRI may detect underlying malignancy.
Paget’s disease of breast is a rare malignancy accounting for 1–3 % cases of breast cancer  and was first described by Sir James Paget in 1874 . Almost 80–90 % of cases are associated with underlying malignancy in the form of ductal carcinoma in situ (DCIS) or invasive breast cancer. The surgical management is individualized and is based on imaging and histopathological findings. In this pictorial essay, we describe the spectrum of imaging findings in biopsy proven cases of Paget’s disease of breast.
Paget’s disease is an unusual breast malignancy where clinical features may be the only sign of cancer during the initial presentation. Imaging plays an important role in evaluating the extent of involvement and in deciding upon patient management.
Paget’s disease of breast is commonly seen in the fifth or sixth decade of life, although it can also be seen in adolescents and the elderly . It is more common in males compared to females  and is usually unilateral . The patients present clinically with changes in the nipple and areolar region in the form of erythema, ulceration, eczematous changes and induration of skin that may be associated with nipple retraction and blood stained nipple discharge . Some of these patients may first approach a dermatologist for treatment of eczema. In cases presenting only with eczema, there is an initial phase of clinical improvement with symptomatic treatment, which causes a delay in diagnosis . A biopsy from the nipple areolar region is necessary to confirm the diagnosis. The differential diagnosis includes allergic contact dermatitis, irritant dermatitis, lichen simplex chronicus and psoriasis . Advanced cases of Paget’s disease may present with a well-demarcated round or ovoid eczema-like plaque that is easily distinguished from the surrounding normal skin. In some cases, a palpable mass may be felt clinically, which is important since approximately 90–95 % of these patients have an underlying invasive cancer . One must not confuse cases of Paget’s disease with inflammatory carcinoma in which there is diffuse erythema involving the entire breast with secondary involvement of nipple and areola.
There are two important theories that explain the possible pathogenesis of Paget’s disease.
Intraepidermal Transformation Theory
According to this theory, Paget cells arise from the epidermis of nipple by degeneration of existing cells or by in-situ transformation (Fig. 1b). This is supported by the fact that in some cases of Paget’s disease, findings are confined to the nipple-areolar region with no underlying DCIS or invasive cancer.
What role does imaging have in the diagnosis of Paget’s disease? Usually, the patient is referred for imaging after a biopsy from the lesion, and this confirms the diagnosis of Paget’s disease. Mammography is the initial radiological investigation for detecting underlying invasive carcinoma or DCIS; however, it may be normal, and ultrasound is performed when mammogram is negative. MRI of breast is performed in patients with negative mammogram and ultrasound findings with no clinically palpable mass.
Magnetic Resonance Imaging
In a case report by Amano et al. , MRI accurately depicted underlying DCIS in a patient without a palpable mass or any positive mammography findings.
The findings on MRI include thickening and enhancement of nipple-areolar complex, an underlying mass, enhancing DCIS or a combination of these findings. Enhancement patterns of the normal nipple-areolar complex are variable, including absent, mild or intense enhancement . However, on comparing the two sides, asymmetric enhancement is noted in the nipple-areolar region with the involved region showing irregular, discoid or irregular enhancement in the case of Paget’s disease . Nipple involvement may also be seen even when there is no clinical suspicion of Paget’s disease . If there is no mass in the sub-areolar region, the entire breast has to be examined to detect any mass that is away from the nipple-areolar region, as the management in such cases is based on imaging findings. Echevarria et al.  discussed the effectiveness of MRI in detecting occult breast cancer associated with Paget’s disease of the nipple-areolar complex, while Peters et al.  discussed the role of MRI as a problem-solving tool in patients with a normal mammogram and no clinically palpable mass. Frei et al.  evaluated pre-operative mammography and MRI findings in nine biopsy proven cases of Paget’s disease and concluded that Paget’s disease of breast with underlying DCIS can be effectively diagnosed by MRI. Though Paget’s disease is predominantly a clinical diagnosis, negative mammography cannot reliably exclude underlying malignancy. Both clinical and imaging findings are complementary to each in order to make a diagnosis .
The treatment of Paget’s disease has been controversial and depends upon the origin of Paget cells. If the epidermotropic theory is believed, then there is an invasive component in the breast and the treatment of choice is mastectomy. The intraepidermal transformation theory, however, says that Paget’s cells form de novo in nipple epithelium, and hence, the treatment of choice in cases confined to the nipple areolar region is resection of nipple-areolar complex followed by local irradiation, making the role of mastectomy controversial.
Paget’s disease of the breast is an uncommon malignancy that is associated with an underlying in situ component or invasive cancer in a large percentage of cases. Surgical management, i.e., breast conservative surgery versus mastectomy, should be decided based on both clinical and imaging findings, including mammography and ultrasound, with MRI playing a crucial role in defining the extent of involvement.
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