Extramammary Paget disease

Claire, Kayla St; Hoover, Alena; Ashack, Kurt; Khachemoune, Amor

doi:10.5070/d3254043591

Cited by 62 publications

(64 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Extension of Paget cells to the adnexal structures may occur. 1,3,4 Penile EMPD of urothelial origin is extremely uncommon and we found only 18 cases published in the English literature. [5][6][7][8][9][10][11][12][13][14][15][16][17][18] We added our patient as case number 19 (Table 1).…”

Section: Discussionmentioning

confidence: 95%

“…It can be focal or multifocal. 1 Paget disease affecting the penoscrotal area was initially described in 1889 by Crocker, who reported it as an eczematous-like lesion in a 60-year-old man. 2 We present an 85-year-old man with penile EMPD of urothelial origin and briefly review previously published cases of EMPD associated with urothelial carcinoma (UC) in English literature.…”

Section: Introductionmentioning

confidence: 99%

“…Clinically, it appears as a plaque lesion with an erythematous or leukoplakic background in regions with abundant apocrine glands such as female external genitalia (65%), perineum (20%), and penis/scrotum (14%). It can be focal or multifocal 1 . Paget disease affecting the penoscrotal area was initially described in 1889 by Crocker, who reported it as an eczematous‐like lesion in a 60‐year‐old man 2…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Penile extramammary Paget disease associated with urothelial carcinoma in situ: Case report and literature review

Tiwari

Kwast

2022

J Cutan Pathol

View full text Add to dashboard Cite

Background: Extramammary Paget disease (EMPD) is an uncommon disease affecting older men and women. Clinically, it appears as a plaque lesion with an erythematous or leukoplakic background in regions with abundant apocrine glands such as female external genitalia, perineum, scrotum, and penis. Methods:We are presenting an 85-year-old patient with recurrent erythematous plaque lesions involving the penis and known to have urothelial carcinoma (UC) in situ of the bladder. A literature review of EMPD secondary to UC has been conducted through PubMed and Google Scholar search engines. Results:The histopathologic examination revealed a proliferation of Paget cells within the surface squamous epithelium. The lesional cells displayed vesicular nuclei, clear cytoplasm, and a positive staining for CK7, CK20, HER2, GATA3 as well as p40, and negative staining for SOX10, CK5/6, and CDX2. The literature review revealed 18 more cases of EMPD associated with UC, including 4 with non-invasive UC. Most of them were treated surgically, but the disease recurred in nine cases and death of disease was reported in at least four patients, all of them associated with invasive UC. Conclusion:The prognosis of penile EMPD seems to be dictated by the stage of the underlying UC.

show abstract

Section: Discussionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Penile extramammary Paget disease associated with urothelial carcinoma in situ: Case report and literature review

Tiwari

Kwast

2022

J Cutan Pathol

View full text Add to dashboard Cite

show abstract

“…In the later stages, nodules or deep ulcers may occur. Although associated symptoms, such as pruritus and tenderness, may develop, around 10% of EMPD patients are asymptomatic [17,18]. EMPD lesions sometimes show subclinical extension, making it difficult for clinicians to determine the clinical borders between EMPD lesions and the surrounding normal skin [19].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…The involved area is then exposed to the appropriate wavelength of light, creating toxic free radicals that destroy tumor cells in the process. Although multiple cases showing antitumor response with PDT have been reported, overall results suggest that PDT may not be a curative treatment but is more beneficial to be used as a palliative treatment to reduce symptoms associated with EMPD lesions [18,124,125]. The side effects of PDT include pain and photosensitivity, which could be severe in some cases [126].…”

Section: Topical Imiquimod (Imq) Creammentioning

confidence: 99%

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Ishizuki

Nakamura

2021

Current Oncology

View full text Add to dashboard Cite

Extramammary Paget’s disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.

show abstract

Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06

et al. 2023

View full text Add to dashboard Cite

BackgroundExtramammary Paget's disease (EMPD) is rare. There are no standard treatments due to its rarity and few clinical trials.MethodsThe objective of this multicenter study was to investigate treatment outcomes of Korean patients with advanced/metastatic EMPD. Data were collected retrospectively from 14 institutions participating in Korean Cancer Study Group (KCSG) Rare Cancer Committee.ResultsA total of 37 patients were identified. Of these 37 patients, 6 received locoregional therapy as a first‐line treatment. In 31 patients who received systemic chemotherapy as a first‐line treatment, platinum‐based chemotherapy (n = 22) achieved an objective response rate (ORR) of 45.5% and a median progression‐free survival (PFS) of 7.89 months. Taxane‐based chemotherapy (n = 8) achieved an objective response rate of 62.5% and median PFS of 9.73 months. In second‐line chemotherapy, platinum‐based chemotherapy (n = 4) had a disease control rate (DCR) of 75.0% and median PFS of 3.45 months. Taxane‐based chemotherapy (n = 8) had a DCR of 75.0% and a median PFS of 8.67 months. Six patients received anti‐human epidermal growth factor receptor 2 (HER2) antibody during first‐ and second‐line chemotherapy. Overall, systemic chemotherapy combined with anti‐HER2 antibody had an ORR of 100% and a median PFS of 13.31 months. The ORR and PFS with systemic chemotherapy combined with trastuzumab was better than platinum‐ and taxane‐based chemotherapy only.ConclusionsDue to its rarity, advanced or metastatic EMPD still has no established standard treatment. Results of our study indicate that the combination of trastuzumab with taxane has longer survival than trastuzumab monotherapy or conventional platinum‐ or taxane‐based chemotherapy.

show abstract

Extramammary Paget disease

Cited by 62 publications

References 37 publications

Penile extramammary Paget disease associated with urothelial carcinoma in situ: Case report and literature review

Penile extramammary Paget disease associated with urothelial carcinoma in situ: Case report and literature review

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06

Contact Info

Product

Resources

About