Not All Cases of Mammary Paget’s Disease are Cytokeratin-7 Positive: A Challenging Diagnosis!

2021 ◽  
pp. 106689692110029
Author(s):  
Kerschen Anja ◽  
Dano Hélène ◽  
Van Eeckhout Pascal ◽  
Marot Liliane ◽  
Van Bockstal Mieke

Mammary Paget’s disease accounts for 1% to 3% of all breast tumors and manifests as a chronic eczematous lesion of the areolar skin. It can occur without any underlying neoplasia or can be present in association with an underlying invasive and/or in situ carcinoma of the breast. The present report describes a challenging nipple punch biopsy showing an infiltration of the lower third to two-thirds of the epidermis by large, ovoid, neoplastic cells. The morphology was consistent with mammary Paget's disease, although immunohistochemistry for cytokeratin-7 (CK7) was repeatedly negative. This resulted in an initial misdiagnosis and, subsequently, a delay in the patient's follow-up. Additional immunohistochemistry for GATA binding protein 3 (GATA3) and human epidermal growth factor receptor 2 (HER2), as well as a second opinion of a breast pathologist, resulted in the diagnosis of mammary Paget's disease. The aim of this article is to raise awareness among pathologists and prevent them from misdiagnosing CK7-negative Paget disease of the breast.

2016 ◽  
Vol 212 (4) ◽  
pp. 279-281 ◽  
Author(s):  
Ugur Ozerdem ◽  
Jennifer M. McNiff ◽  
Fattaneh A. Tavassoli

2020 ◽  
Vol 30 (11) ◽  
pp. 1672-1677 ◽  
Author(s):  
Michele Bartoletti ◽  
Roberta Mazzeo ◽  
Marco De Scordilli ◽  
Anna Del Fabro ◽  
Maria Grazia Vitale ◽  
...  

BackgroundInvasive vulvar Paget’s disease with over-expression of the human epidermal growth factor receptor 2 (HER2) protein is potentially suitable for targeted therapy, especially in a metastatic setting where no effective treatments are available.MethodsFour consecutive patients with HER2 positive advanced vulvar Paget’s disease, treated with weekly trastuzumab (loading dose 4 mg/kg, then 2 mg/kg) and paclitaxel (80 mg/m2) followed by 3-weekly trastuzumab maintenance (6 mg/kg), are reported.ResultsMedian age and follow-up of patients were 62.5 years (45–74) and 16 months (6-54), respectively. Complete or partial responses were observed in all patients. Median time to response was 3 months (range 2–4), while median duration of response was 10 months (range 2–34). Case 1 presented with pulmonary and lymph nodes involvement. She experienced a radiological complete response after 24 treatment administrations, and a progression-free survival of 36 months. At disease progression, treatment re-challenge achieved partial response. She is currently receiving treatment with trastuzumab–emtansine. Case 2 was a 74-year-old woman who developed pulmonary metastasis after first-line cisplatin treatment. She had a partial response and a progression-free survival of 10 months. Case 3 had inguinal and para-aortic lymphadenopathy in complete response after 18 treatment administrations. She developed brain metastasis while receiving trastuzumab maintenance. Case 4 was treated for locally advanced disease and experienced a subjective benefit with relief in perineal pain and itching. No unexpected treatment-related side effects were reported.ConclusionsAdvanced vulvar Paget’s disease is a rare disorder and no standard treatment is available. In the sub-group of HER2 positive disease, weekly paclitaxel–trastuzumab appears to be active and safe, and may be considered a therapeutic option in these patients.


2014 ◽  
Vol 67 (11) ◽  
pp. 1010-1012 ◽  
Author(s):  
Duaa Saeed ◽  
Sami Shousha

AimsToker cells are clear cells present in the squamous epithelium of the nipple of some women. In contrast to squamous epithelium, they are cytokeratin 7 (CK7) positive. The origin of these cells is not completely understood. It has been suggested that they may represent abortive glands or migratory ductal cells; and may be precursors of Paget's disease of the nipple. Our aim was to investigate the incidence and distribution of Toker cells and their relationship with lactiferous ducts.MethodsWe examined nipple sections from 100 consecutive mastectomies performed at Charing Cross hospital. New sections were stained for CK7 using the immunoperoxidase technique.ResultsToker cells were identified in 11 cases. They were always clustered within the squamous epithelium superficial to sebaceous glands with no relationship with lactiferous ducts. Two cases in the study had Paget's disease and these were not associated with underlying sebaceous glands.ConclusionsThis study suggests that Toker cells are more likely to be developmentally related to sebaceous glands rather than lactiferous ducts. This raises doubts about the presence of a relationship between Toker cells and the common forms of Paget's disease, as the latter are commonly seen in association with ductal carcinoma in situ (DCIS) involving underlying lactiferous ducts. Toker cells, however, may be related to a less common form of Paget's disease which is not associated with underlying DCIS.


2020 ◽  
Vol 41 (3) ◽  
pp. 232-237
Author(s):  
Shoukat Arain ◽  
Maria Arafah ◽  
Emad Said Raddaoui ◽  
Asma Tulba ◽  
Fatimah Alkhawaja ◽  
...  

10.3823/2546 ◽  
2018 ◽  
Vol 11 ◽  
Author(s):  
Debora Bacellar Cruz Nunes ◽  
Adriana Kamilly Leitão Pitman Machado ◽  
Michelle Fonseca Goiabeira ◽  
Samira Oliveira Silveira ◽  
Yandra Sherring Einecke ◽  
...  

An atopic breast eczema is usually bilateral, with intense pruritus and good response to corticosteroids. The differential diagnosis with mammary Paget’s disease is fundamental, mainly in unilateral clinical manifestations, since it is frequently associated to adjacent breast tumors. The aim of the present report was to present a clinical condition of an atopic breast eczema through an exuberant and unilateral clinical presentation, emphasizing the need of the differential diagnosis for malignant diseases at this location.


2021 ◽  
pp. 1242-1247
Author(s):  
Wataru Goto ◽  
Shinichiro Kashiwagi ◽  
Yuko Kawano ◽  
Asuka Komoda ◽  
Sae Ishihara ◽  
...  

Mammary Paget’s disease is a rare malignancy. Mastectomy or breast-conserving surgery has been considered as the standard treatment, while there have been few reports of neoadjuvant chemotherapy (NAC). A 53-year-old woman with erythema and skin ulceration of the left breast was admitted to our institution. Breast examinations revealed left invasive ductal carcinoma (cT1bN0M0, cStage I), and a punch biopsy of the left mammary erythema indicated Paget’s disease (cTisN0M0, cStage0). The patient received NAC because of multiple lesions. Consequently, the breast tumor clinically disappeared, and the erythema improved. These outcomes made it easier to perform surgery (left mastectomy and sentinel node biopsy). Histopathological examination revealed no residual cancer cells in either the mammary gland or breast skin, and no metastasis was found in the sentinel lymph node. Therefore, NAC may be a useful treatment for mammary Paget’s disease.


2019 ◽  
Vol 28 (2) ◽  
pp. 210-215 ◽  
Author(s):  
Samantha A. Moore ◽  
Hollis M. Notgrass ◽  
Travis W. Vandergriff ◽  
Sunati Sahoo

Mammary Paget’s disease is rare and comprises about 0.62% of all breast cancer cases, only 1.65% of which occur in male patients. This case report involves a 76-year-old man who presented to his primary care physician with an itching, scaly, unilateral lesion involving the nipple skin. He underwent wide local excision of the lesion for a diagnosis of Bowen’s disease (squamous cell carcinoma in situ). Histologic examination of the specimen revealed mammary Paget’s disease with ductal carcinoma in situ in the underlying breast tissue. A panel of immunohistochemical stains revealed the Paget cells to be positive for cytokeratin 7, MUC1, GATA3, and androgen receptor and negative for cytokeratins 5/6, p63, SOX10, and MART-1/Melan-A. Paget cells were also negative for estrogen receptor and progesterone receptor, and positive for HER2/neu. However, the underlying ductal carcinoma in situ was positive for both estrogen receptor and progesterone receptor and negative for HER2/neu. This discordance, supported by the current literature, suggests an alternative etiology for Paget’s disease in certain cases that cannot be explained by the well-established epidermotropic and transformative theories of Paget’s disease evolution.


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