Extramammary Paget’s Disease in Males: Survival Outcomes in 495 Patients

Herrel, Lindsey A.; Weiss, Aaron D.; Goodman, Michael; Johnson, Timothy V.; Osunkoya, Adeboye O.; Delman, Keith A.; Master, Viraj A.

doi:10.1245/s10434-014-4139-y

Cited by 68 publications

(53 citation statements)

References 12 publications

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“…Although most primary EMPDs are intraepithelial, approximately 4% to 20% primary vulvar EMPDs [1–4, 9, 13, 15, 16] and 26% to 61% primary penoscrotal EMPDs were invasive at the time of presentation [5, 7, 8, 10, 58–61]. Some of these invasive adenocarcinomas arise from the intraepithelial EMPD (type Ib primary EMPDs) whereas others are underlying adenocarcinomas which showed secondary epidermotropism (type Ic primary EMPDs) [3, 6, 8, 9, 14–16].…”

Section: Discussionmentioning

confidence: 99%

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GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15

et al. 2017

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BackgroundGATA-binding protein 3 (GATA3) has been identified as a sensitive marker for breast carcinoma but its sensitivity in primary genital extramammary Paget diseases (EMPDs) has not been well studied.MethodsHere we investigated immunohistochemical expression of GATA3 in 72 primary genital EMPDs (35 from female, 37 from male; 45 with intraepithelial disease only, 26 with both intraepithelial disease and invasive adenocarcinoma including 14 also metastasis, 1 with metastatic adenocarcinoma only for study). We also compared GATA3 to gross cystic disease fluid protein 15 (GCDFP15) for their sensitivity.ResultsPositive GATA3 staining was seen in all 71 (100%) intraepithelial diseases, 25/26 (96%; female 10/10, male 15/16) invasive adenocarcinomas and 14/15 (93%; female 3/3, male 11/12) metastatic adenocarcinomas, respectively. Positive GCDFP15 staining was seen in 46/71 (65%; female 28/34 or 82%, male 18/37 or 49%) intraepithelial diseases, 20/26 (77%; female 9/10, male 11/16) invasive adenocarcinomas, and 12/15 (80%; female 2/3, male 10/12) metastatic adenocarcinomas, respectively (GATA3 versus GCDFP15: p < 0.01 for both intraepithelial disease and invasive adenocarcinoma, p = 0.28 for metastatic adenocarcinoma). In positive-stained cases, GATA3 stained more tumor cells than GCDFP15 (79% versus 25% for intraepithelial disease, 71% vs 34% for invasive adenocarcinoma, 73% vs 50% for metastatic adenocarcinoma, p < 0.01 for all 3 components).ConclusionsOur findings indicate that GATA3 is a very sensitive marker for primary genital EMPDs and is more sensitive than GCDFP15.

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Section: Discussionmentioning

confidence: 99%

“…Among patients with invasive EMPDs (type Ib and type Ic), 20% to 40% had lymph node metastasis [4–7, 9, 11]. Up to 17% to 50% patients with invasive EMPDs also develop concurrent or subsequent distant metastasis [4, 5, 7, 9–12]. …”

Section: Introductionmentioning

confidence: 99%

GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15

et al. 2017

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“…It has been reported that the incidence of EMPD is higher among Asians than white patients based on the database of Surveillance, Epidemiology and End Results (SEER). 4 Ohara et al 5 reported that LNM in bilateral inguinal regions has a poor prognosis. In that study, the number of cases of bilateral lateral LNM was limited; however, there was no difference in prognosis between bilateral and unilateral inguinal LNM.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: A single‐institution study

Tsutsui

Takahashi²,

Muto³

et al. 2020

The Journal of Dermatology

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Extramammary Paget's disease (EMPD) often invades the dermis and metastasizes to the lymph nodes. Patients with EMPD associated with lymph node metastases have poor prognosis; to date, effective treatment has not yet been established. Lymph node dissection, aiming to control the local disease, is a standard form of management for EMPD patients with lymph node metastases (LNM). We investigated the clinical and pathological features, treatment strategies and prognostic factors of patients with metastatic EMPD who underwent lymph node dissection. We retrospectively evaluated 38 cases of extramammary Paget's disease with lymph node metastasis over 10 years. All patients underwent wide resection of the primary lesion and lymph node dissection. Univariate analysis revealed the number of metastatic nodes and lymphadenopathy as prognostic factors. In multivariate analysis, the number of metastatic lymph nodes retained statistical significance (hazard ratio, 35.3; 95% confidence interval, 3.23-387.0; P = 0.003). The 5-year survival rate was 100% and 19.1% in patients with two or less LNM and with three or more LNM, respectively. In patients with three or more LNM, the 5-year survival rate after adjuvant radiation therapy was better than that after surgery alone (75% vs 0%). In conclusion, patients with two or less LNM can be expected to have long-term survival with lymph node dissection only, while patients with three or more LNM may require adjuvant radiation therapy to improve prognosis. These results suggest that lymph node dissection may be a strategy to treat EMPD with regional LNM. METHODS PatientsThis retrospective analysis examined a prospectively maintained database of 89 EMPD patients with dermal invasion who were treated at

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“…Extramammary Paget's disease (EMPD) is an intraepithelial adenocarcinoma which predominantly affects areas with a high apocrine gland concentration, including the vulva, perineal, perianal, scrotal, penile and axillae skin. [1][2][3] EMPD lesions are notorious for its chronic relapsing clinical course and unbearable clinical symptoms such as itching, skin ulcer and bleeding. [2,4] The standard treatment for EMPD is often extensive surgical excision followed by plastic reconstruction, which frequently leaves anatomical and functional impairments, and results in a signi cantly diminished quality of life.…”

Section: Introductionmentioning

confidence: 99%

“…[12,13,18] In multiracial country like the United States, the incidence of EMPD showed ethnic disparity, the incidence in black males was nearly four times lower and in Asians/Paci c islander males four times higher than that in Caucasian males between 1973 and 2009. [1] No national epidemiological studies are available to estimate the prevalence and incidence of EMPD in Asian countries, including China.…”

Section: Introductionmentioning

confidence: 99%

Prevalence of Extramammary Paget's Disease in Urban China: A Population-based Study

Yin

Wang

et al. 2020

Preprint

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Background Extramammary Paget’s disease (EMPD) is an intraepithelial adenocarcinoma. The chronic relapsing clinical course and unbearable clinical symptoms of EMPD usually result in a markedly diminished quality of life. No national data are available on descriptive epidemiology of EMPD in China, the most populous country over the world. This population-based study aimed to estimate the prevalence and associated sex and age patterns of EMPD in China. Methods This study was conducted using data from China’s Urban Employee Basic Medical Insurance and Urban Resident Basic Medical Insurance, covering approximately 0.43 billion Chinese urban residents in 2016. Patients with EMPD were identified based on the diagnostic names and codes in claim data. Results A total of 53 males and 31 females with EMPD were found. The crude prevalence in 2016 was 0.04 per 100 000 population [95% confidence interval (CI): 0.02 to 0.06], ranging from 0.01 (95% CI: 0.00 to 0.02) in North or Northeast China to 0.08 (95% CI: 0.03 to 0.16) in Southwest China. The rates were higher in males (0.05, 95% CI: 0.03 to 0.08) compared with females (0.03, 95% CI: 0.02 to 0.05). The mean age of patients was 65.87 (standard deviation: 14.21) years, with the peak prevalence appeared in patients aged 70–79 (0.28, 95% CI: 0.16 to 0.42). Conclusions The prevalence of EMPD was markedly lower than those in the United States and Europe, and varied across regions in China. Chinese patients were much younger, with significant male predominance. Further study is warranted to examine potential pathophysiologic mechanism.

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Extramammary Paget’s Disease in Males: Survival Outcomes in 495 Patients

Abstract: Survival in men with EMPD is lower among those with distant disease and primary tumors located in the perianal region. The reasons for increasing EMPD incidence over time and for the racial disparities in disease occurrence require further study.

Cited by 68 publications

References 12 publications

GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15

GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15

Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: A single‐institution study

Prevalence of Extramammary Paget's Disease in Urban China: A Population-based Study

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