Paget disease of the breast

Uncommon case
of mammary Paget’s disease (MPD) after ductal carcinoma in situ (DCIS) treated with breast-sparing surgery and brachytherapy. MRI 03/2010: occasional spontaneous nipple discharge. Extended duct with protracted enhancement in the left-upper quadrant, up to the nipple, small adjacent foci. DD: intraductal papilloma, intraductal malignancy. BIRADS IV (a) Dynamic-MRI (b) subtracted-MRI 4-minutes-delay.
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Uncommon case
of mammary Paget’s disease (MPD) after ductal carcinoma in situ (DCIS) treated with breast-sparing surgery and brachytherapy. 02/2011 Multimodal imaging: history of spontaneous discharge, slightly retractile scar tissue. MRI: postoperative changes: BIRADS II. (a) Dynamic-MRI 4-minutes-delay, (b) subtracted MRI after 4 minutes, (c) mammography BIRADS II, (d) US scar. BIRADS II.
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Uncommon case
of mammary Paget’s disease (MPD) after ductal carcinoma in situ (DCIS) treated with breast-sparing surgery and brachytherapy. (a) Mammography 01/2013 BIRADS II. (b) US 03/2013: top left and right, white arrows: scar. bottom left, yellow arrows: two contiguous hypoechoic, well-defined focal lesions. Histology: MPD. Axillary lymph node (bottom right, asterisk): normal. BIRADS IV
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Uncommon case
of mammary Paget’s disease (MPD) after ductal carcinoma in situ (DCIS) treated with breast-sparing surgery and brachytherapy. MRI 03/2013: shows a new onset suspicious lesion in the left subareolar region with retraction of the nipple. BIRADS IV. (a) Dynamic and (b) subtracted after 2 minutes. (c) Fat saturated sequence after 4 minutes.
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Scaly,
erythematous, crusty, and thickened plaque on the nipple of Paget Disease of the Breast.
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Multimodality
approach to the nipple-areolar complex: a pictorial review and diagnostic algorithm. Paget’s disease (I). A 56-year-old woman with right nipple retraction. a 2D mammogram shows a spiculated retroareolar mass in the right breast with nipple retraction and skin thickening; b on ultrasound, it is seen as a solid lesion with ill-defined borders. c MRI shows the retroareolar lesion extending to the nipple-areolar complex. Histologic study revealed infiltrating ductal carcinoma extending to the epidermis
insightsimaging.springeropen.com • CC-by-4.0
Multimodality
approach to the nipple-areolar complex: a pictorial review and diagnostic algorithm. Paget’s disease (II). An 81-year-old woman with an ulcerated lesion in her left nipple. a Synthesized 2D mammogram shows slight skin thickening in the left nipple-areolar complex (arrows). b MRI shows differential pathologic enhancement of the left nipple-areolar complex. c, d Hematoxylin-eosin-stained punch-biopsy specimen (× 0.3 and × 4, respectively) shows ductal carcinoma with extensive infiltration of the nipple and ulceration of the epidermis (Paget’s disease)
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Radiopaedia • CC-by-nc-sa 3.0 • de

Paget disease of the breast, which is also known as Paget disease of the nipple, has traditionally been described as a form of breast malignancy characterized by infiltration of the nipple epidermis by malignant cells. Although most cases have underlying focus or foci of in situ or invasive carcinomas, some cases of Paget disease are confined to the skin of the nipple-areola without underlying neoplastic foci.

Epidemiology

The condition can represent 1-5% of breast malignancies. The average age at diagnosis is around the 6 decade (53-59 years).

Clinical presentation

Eczematous appearing changes of the nipple include reddening, scaling, hyperkeratosis, and crusting of the nipple surface.

Clinical staging

Mammary Paget disease can be classified into four clinical stages:

  • stage 0: lesion is confined to the epidermis, without underlying ductal carcinoma in situ (DCIS) of the breast
  • stage 1: associated with DCIS just beneath the nipple
  • stage 2: associated with extensive DCIS
  • stage 3: associated with invasive ductal carcinoma

Pathology

In most cases of Paget of the breast, there are malignant ductal cells that extend to the nipple surface through the terminal lactiferous ducts.

Malignant epithelial (Paget) cells infiltrate and proliferate in the epidermis, causing an eczema-like rash of the nipple and the areolar skin. There are several histologic variants of Paget disease including:

  • adenocarcinoma-like cell type
  • spindle cell type
  • anaplastic cell type
  • acantholytic cell type
  • pigmented cell type

Radiographic features

Mammography

Mammary Paget disease can be undetectable on mammography in ~50% of cases . Features that may be evident on mammography include skin thickening, nipple retraction, subareolar or more diffuse malignant microcalcifications, and discrete subareolar mass(es).

Breast MRI

MR imaging may play an important role in the selection of patients with Paget disease for breast-conserving therapy if there is no clinical or mammographic evidence of breast carcinoma. Abnormal nipple enhancement and linear clumped enhancement indicative of DCIS in association with Paget disease.

Treatment and prognosis

Traditionally, treatment has been a mastectomy with nodal dissection. However, with MRI, breast-conserving therapy is more frequently being used.

History and etymology

Paget disease of the breast is named after Sir James Paget (1814-1899), the British surgeon and pathologist, who first described it in 1874 .

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Paget disease of the nipple:
Mammakarzinom
 
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