Paget’s disease of the breast – observational retrospective study of the past 20 years

2021 ◽  
Vol 100 (4) ◽  
Keyword(s):  
Local Recurrence ◽  
Imaging Techniques ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Her2 Positive ◽  
Rare Type ◽  
Areolar Complex ◽  
Initial Symptoms ◽  
Careful Clinical Examination ◽  
Associated Malignancy

Introduction: Paget‘s disease of the breast (PD) is a rare type of carcinoma that affects the skin of the nipple-areolar complex. Unresolved issues exist regarding its diagnosis and therapy. The aim of the study was to gather data on how the therapy of the disease is approached in clinical practice, and to formulate current diagnostic and therapeutic recommendations. Methods: Retrospective evaluation of data from medical records of patients with PD who underwent surgery at our department between 2001 and 2020. The data was evaluated using basic statistical methods. Results: Sixty four female patients with the mean age of 62.5 years. In 58 women, PD was confirmed before surgery, with the median of 20 weeks from initial symptoms to diagnosis. Forty seven of the patients were operated for presumed isolated PD; in 38 cases, histopathological evaluation of the specimen revealed an associated malignancy in the mammary gland. Primary breast-conserving surgery (BCS) was performed in 46 patients; surgical revision was indicated in 17 cases. In 6 patients with PD associated with non-invasive breast cancer treated by BCS without radiotherapy (RT), a local recurrence appeared in 3 cases, which is significantly more compared to the group of patients undergoing total mastectomies (p=0.032). No local recurrence appeared in 9 cases of isolated PD treated by BCS, including 6 patients without RT. The tumors associated with PD were mostly ER-negative (44/57) and HER2-positive (22/25). Conclusion: In cases where PD is suspected, careful clinical examination and the use of available diagnostic imaging techniques including MRI are appropriate. BCS without RT is not an adequate oncological therapy where an associated malignancy of the breast is found.

Thirty Year Old Female Presented with Nipple Destruction: A Case Report

2019 ◽  
Vol 11 (1) ◽  
pp. 81-83
Author(s):  
Md Mustafizur Rahman ◽  
Nadim Ahmed ◽  
Sami Ahmad ◽  
Shoaeb Imtiaz Alam ◽  
Mohammad Rashedul Hassan ◽  
...  
Keyword(s):  
Invasive Carcinoma ◽  
Paget’S Disease ◽  
Radical Mastectomy ◽  
Paget's Disease ◽  
College Hospital ◽  
Rare Type ◽  
Medical College ◽  
Ductal Cell ◽  
Areola Complex

Paget’s disease of the breast is a rare type of cancer of the nipple–areola complex and that is often associated with an underlying in situ or invasive carcinoma. It is often misdiagnosed as eczema of breast and treatment is delayed. Here we present a case where a 30 year old female presented with itching ulceration and destruction of her left nipple. She was treated initially by local physicians by applying local ointments but as her condition did not improve she was admitted to department of surgery Shaheed Suhrawardy Medical College hospital where she was diagnosed as Paget’s disease with infiltrating ductal cell carcinoma. She underwent modified radical mastectomy with axillary clearance and referred to oncology department for further management. J Shaheed Suhrawardy Med Coll, June 2019, Vol.11(1); 81-83

scholarly journals Human epidermal growth factor receptor-2 (HER2) is a potential therapeutic target in extramammary Paget’s disease of the vulva

2020 ◽  
Vol 30 (11) ◽  
pp. 1672-1677 ◽  
Author(s):  
Michele Bartoletti ◽  
Roberta Mazzeo ◽  
Marco De Scordilli ◽  
Anna Del Fabro ◽  
Maria Grazia Vitale ◽  
...  
Keyword(s):  
Epidermal Growth Factor Receptor ◽  
Paget’S Disease ◽  
Growth Factor Receptor ◽  
Progression Free Survival ◽  
Complete Response ◽  
Paget's Disease ◽  
Her2 Positive ◽  
Free Survival ◽  
Vulvar Paget’S Disease ◽  
Epidermal Growth

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.

scholarly journals Tumor Wide Horizontal Invasion Predicts Local Recurrence for Scrotal Extramammary Paget’s Disease

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Lujia Wang ◽  
Chenchen Feng ◽  
Minwei Zhou ◽  
Zhongwen Zhou ◽  
Guanxiong Ding ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Extramammary Paget's Disease

scholarly journals Paget's Osteosarcoma — no Cure in Sight

Sarcoma ◽  
1999 ◽  
Vol 3 (3-4) ◽  
pp. 191-192 ◽  
Author(s):  
Phillip J. Shaylor ◽  
David Peake ◽  
Robert J. Grimer ◽  
Simon R. Carter ◽  
Roger M. Tillman ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Limb Salvage ◽  
Poor Prognosis ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Recurrence Rates ◽  
Curative Intent ◽  
Sarcomatous Change ◽  
Incurable Disease ◽  
Historical Series

PurposePaget' s osteosarcoma has a fearful reputation with a quoted survival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treated over the last 25 years using modern staging and limb salvage techniques to see if there had been any improvement in survival.Subjects:We identified 26 patients on the Royal Orthopaedic Hospital Oncological database with a diagnosis of sarcoma secondary to Paget's disease.Results:The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years.The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb-sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months.There was no difference in survival between amputation and limb salvage.Discussion:The development of sarcomatous change in Paget's disease is well recognised. It represents an important segment of primary bone tumours in patients over 40 years of age.The prognosis is appalling. Indeed only 15 of 368 cases (4%) from a number of historical series have survived more than 5 years. Our results are similarly disappointing with no survivors at 5 years despite modern methods of management of bone tumours.While there is no difference in local recurrence rates or survival between limb reconstruction and limb ablation the poor prognosis for both means that neither can be recommended at present. Sarcomatous change in Pagetoid bone should therefore be regarded as a different disease to primary osteosarcoma. It remains an incurable disease with a poor prognosis.

scholarly journals Practical consensus recommendatons for Paget’s disease in breast cancer

2018 ◽  
Vol 07 (02) ◽  
pp. 083-086 ◽  
Author(s):  
A. Gaurav ◽  
V. Gupta ◽  
R. Koul ◽  
S. Dabas ◽  
R. Sareen ◽  
...  
Keyword(s):  
Invasive Carcinoma ◽  
Treatment Options ◽  
Paget’S Disease ◽  
Practical Experience ◽  
Disease Diagnosis ◽  
Paget's Disease ◽  
Expert Group ◽  
Rare Type ◽  
Areola Complex

AbstractPaget's disease of the breast is a rare type of cancer of the nipple–areola complex and that is often associated with an underlying in situ or invasive carcinoma. Diagnosis and treatment of Paget's disease is controversial. Expert oncologists discuss on the update on the approaches of Paget's disease diagnosis and its treatment options. This expert group used data from published literature, practical experience and opinion of a large group of academic oncologists to arrive at this practical consensus recommendations for the benefit of community oncologists.

scholarly journals Associated malignancy in extra-mammary and mammary Paget’s Disease: A retrospective analysis of single institute experience

2017 ◽  
Vol 28 ◽  
pp. x179
Author(s):  
P. Thirasastr ◽  
V. Sriuranpong ◽  
N. Poovorawan ◽  
T. Atikankul ◽  
N. Parinyanitikul
Keyword(s):  
Retrospective Analysis ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Associated Malignancy ◽  
Mammary Paget’S Disease

scholarly journals Paget’s Disease of the Breast Presenting as a Local Recurrence Following Breast-conserving Surgery

2016 ◽  
Vol 38 (1) ◽  
pp. 29-31
Author(s):  
Haldun Kar ◽  
Necat Cin ◽  
Cengiz Tavusbay ◽  
Kursat Yemez ◽  
Yasin Peker ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Paget’S Disease ◽  
Breast Conserving Surgery ◽  
Paget's Disease
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