Prognostic Analysis of Patients With Extramammary Paget Disease Treated With Conservative Excision.

BACKGROUND: Extramammary Paget disease (EMPD) is a malignant skin tumor with a relatively good prognosis. The standard treatment is wide local resection or Mohs micrographic surgery. However, conservative excision may be a better option when radical wide local excision is difficult to perform due to the patients' mental or physical condition. There have been no studies on the prognosis of patients with EMPD who underwent conservative excision. OBJECTIVE: To compare the prognosis of conservative excision cases to wide excision cases of EMPD. MATERIALS AND METHODS: The authors retrospectively analyzed the clinical data of 69 cases of EMPD without metastases to lymph nodes or organs (11 cases treated with conservative excision, 58 cases treated with wide local excision) who underwent resection of the primary tumor from 2002 to 2022 in the Department of Dermatology at Hokkaido University Hospital. RESULTS: The log-rank test showed no significant differences in overall survival or metastasis-free survival between the wide excision group and the conservative excision group, although conservative surgery was often chosen in elderly patients or patients with lower performance status. CONCLUSION: This study suggests that conservative surgery should be considered as a treatment option for EMPD.

Extramammary Paget's Disease of the Vulva and Concomitant Premalignant/Malignant Vulvar Lesions: A Potential Challenge in Diagnosis and Treatment.

The aim of the present study was to evaluate the incidence of concomitant vulvar cancers or premalignant lesions in women surgically treated for extramammary Paget's disease of the vulva (EMPDV) through a multicenter case series. The medical records of all women diagnosed with and treated for EMPDV from January 2010 to December 2020 were retrospectively analyzed. Women with EMPDV and synchronous vulvar cancer, vulvar intraepithelial neoplasia (VIN) and/or lichen sclerosus (LS) at the histology report were included in the study. A total of 69 women eligible for the present study were considered. Concomitant vulvar lesions occurred in 22 cases (31.9%). A total of 11 cases of synchronous VIN (50%) and 14 cases (63.6%) of concomitant LS were observed. One patient (4.5%) had synchronous vulvar SCC (FIGO stage 1B). Women with EMPDV and concomitant premalignant/malignant vulvar lesions had a significantly higher rate of invasive EMPDV and wider lesions with an extravulvar involvement. The specific meaning of the association between EMPDV, VIN, SCC and LS remains unclear. The potential overlapping features between different vulvar lesions highlight the importance of dedicated gynecologists and pathologists in referral centers.

Identification of Risk Factors of Recurrence Among Patients With Vulvar Paget Disease Treated With Conservative Surgery.

OBJECTIVES: Vulvar Paget disease (VPD) is a benign disease with high recurrence rates. Standard treatment involves conservative surgery with wide local excision of the lesion. The purpose of the present study is to identify factors that increase the risk of relapse. MATERIALS AND METHODS: We conducted a retrospective study and included patients treated with conservative surgery for noninvasive VPD. Cox regression analysis was carried out to assess the independent effect of age, presence of positive margins, tumor size greater than 4 cm, bilateral lesions, and compositive morbidity and pathology on recurrence free survival. Post hoc power analysis was performed with the G-power tool using an α error of 0.05. RESULTS: Overall, 39 patients were included with a median age of 70 years (46-85 years). Of those, 19 patients relapsed within a median duration of 30.5 months (5-132 months). Twelve patients (63%) experienced at least a second relapse. The presence of composite comorbidity significantly affected the interval to recurrence (30.09 vs 71.80 months, p = .032). Univariate Cox regression analysis revealed that the presence of composite pathology features was indicative of a higher risk of recurrence (hazard ratio = -3.71, p = .024). The sample size did not allow for adequate power for this latter finding. Microscopically involved tumor margins and tumor size greater than 4 cm did not predict patients at risk of experiencing relapsing disease. CONCLUSIONS: Patients with noninvasive VPD experience high relapse rates. The presence of concurrent benign vulvar pathology may increase these rates, although larger sample sizes are needed to ascertain our findings.

Trends in incidence and survival of patients with vulvar cancer in an Asian country: Analysis of the Korean Central Cancer Registry 1999-2018.

OBJECTIVE: To report 20-year trends in incidence and survival of vulvar cancer in Korea. METHODS: Using data from the Korean Central Cancer Registry, age-standardized incidence rates (ASRs) and annual percentage changes (APCs) were calculated. Net survival (NS) was estimated by the Pohar-Perme method, and conditional net survival (CNS) was calculated. RESULTS: A total of 2221 patients was diagnosed with vulvar cancer during the 1999-2018 period, with an ASR of 0.32 per 100,000 person-years. Among the cases, 51.4% were squamous cell carcinoma (SqCC), 21.3% were Paget disease, and 8.6% were basal cell carcinoma (BCC). There was an increase in incidence for all vulvar cancer (APC 2.4%, 95% CI 1.8-3.0). However, although BCC (APC 7.0%, 95% CI 3.3-10.8) and Paget disease (APC 5.9%, 95% CI 4.2-7.6) increased, SqCC did not (APC 0.2%, 95% CI -0.8-1.2). There was an increase in incidence in all age groups. The 5Y NS was 74.0% overall, and it did not improve significantly during the study period. The 5Y CNS of vulvar cancer increased continuously with time survived: from 74.0% (71.4-76.4) at baseline to 98.1% (95% CI, 85.4-99.8) at 5 years after diagnosis. CONCLUSIONS: The incidence of vulvar cancer in Korea showed a different pattern from those in the US and Europe: SqCC incidence was relatively low and remained stable, but the incidence of BCC and Paget's disease increased. Survival did not improve in the past two decades. Patients can be considered cured after surviving for 5 years.

Survival analysis of extramammary Paget's disease (EMPD) in a tertiary hospital in Taiwan.

BACKGROUND: This study aimed to investigate the survival analysis of extramammary Paget's disease (EMPD) in a Taiwanese population and to provide data for comparison with other studies in various locations and racial populations. METHODS: We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Independent variables included patients' demographic data, concurrent malignancy (i.e., non-EMPD-related cancers), tumor size, distant metastasis, and surgery and/or radiation. RESULTS: Of all the 63 patients, 8 cases were excluded. A total of 43 patients (78.18%) were male, and 12 were female, with a mean age of 72.67 years (range 44-89 years). The most common affected anatomic site was the penoscrotal region (22 patients, 40.00%), followed by the perianal and perineal regions (17 patients, 30.91%). Among the 55 patients, 41 patients (74.55%) were diagnosed with at least one underlying disease, whereas the most common underlying disease was cardiovascular disease (30 patients, 54.55%). The overall survival rate was 80.00% at 36 months and 65.45% at the end of follow-up. EMPD with deep dermal invasion was a significant poor prognostic factor of overall survival in cause-specific hazard model (sub-hazard ratio (HR) 5.167, p = 0.0015, 95% confidence interval (CI) 1.876-14.230). Patients with regional metastasis or distant metastasis had poorer prognosis of 5-year survival (sub-HR 4.513, p = 0.0028, CI 1.683-12.103). The limitations of this study include its retrospective nature and sample size. CONCLUSIONS: In our series, EMPD with metastasis and deep dermal invasion was the significant harmful factors in both overall 5-year survival and 5-year recurrence-free survival. The surgical excision is not associated with a low risk of local recurrence or overall survival, and long-term follow-up is still needed.

Prevalence of extramammary Paget's disease in urban China: a population-based study.

BACKGROUND: Extramammary Paget's disease (EMPD) is an intraepithelial adenocarcinoma. The chronic relapsing clinical course and unbearable clinical symptoms of extramammary Paget's disease 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) 0.02-0.06], ranging from 0.01 (95% CI 0.00-0.02) in North or Northeast China to 0.08 (95% CI 0.03-0.16) in Southwest China. The rate was higher in males (0.05, 95% CI 0.03-0.08) compared with females (0.03, 95% CI 0.02-0.05). 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 0.16-0.42). 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 studies are warranted to examine potential pathophysiologic mechanism.

Genital premalignant and malignant diseases: a retrospective study of male genital skin biopsies.

BACKGROUND: Genital skin malignancies are a rare entity encountered by clinicians that may result in significant morbidity and mortality. Lack of familiarity or expertise in this area among clinicians can delay appropriate management of these conditions and may result in disease progression. METHODS: We performed a retrospective descriptive cohort study of male patients who received a genital skin biopsy reported by one major dermatopathology laboratory between January 2017 and December 2018 with a histological diagnosis of a premalignant or malignant condition. Patient age, type of clinician, clinical notes, genital site, type of biopsy performed, and histopathological diagnosis were evaluated. RESULTS: Of the 1525 male genital skin biopsies available for analysis, 5% (74/1525) were premalignant or malignant diseases. These included penile intraepithelial neoplasia (PeIN) (42/74, 57%), followed by invasive squamous cell carcinoma (SCC) (11/74, 15%), Bowenoid papulosis (BP) (9/74, 12%), basal cell carcinoma (8/74, 11%), malignant melanoma (2/74, 2.7%), extramammary Paget's (1/74, 1.4%), and metastatic cutaneous deposits (1/74, 1.4%). PeIN and BP most commonly affected the penile shaft ([18/42] 43% and [4/9] 44%, respectively), invasive SCCs most commonly affected the glans penis (4/11, 36%), and all BCCs (8/8, 100%) were located on the scrotum. Invasive SCCs were most biopsied by urologists, BP was most biopsied by dermatologists, and PeINs and BCCs were most biopsied by general practitioners. CONCLUSIONS: A variety of genital malignancy types were found in males. A greater understanding of male genital premalignant and malignant conditions may help guide education and further research in this area.

Extramammary Paget disease of the vulva.

Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is characterized by slow grow and high recurrence rates. This article reviews the epidemiological, clinical, morphological, genetic and treatment features of EMPD of the vulva reported in recent years.

Clinical characteristics and risk factors of invasion in extramammary Paget's disease of the vulva.

PURPOSE: This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget's disease of the vulva (EPDV). METHODS: We performed a retrospective analysis of patients who were initially diagnosed with EPDV in Fudan University Shanghai Cancer Center between May 2006 and March 2019. RESULTS: Thirty-eight patients were initially diagnosed with EPDV in our institution. Among them, 29 had intraepithelial EPDV, 8 had intraepithelial EPDV with stromal invasion, and 1 had an underlying vulvar adenocarcinoma. In total, 8 (21%) patients had 12 recurrences. Of these eight patients, four had one recurrence, while other four had two recurrences. Intraepidermal EPDV recurred nine times, while intraepidermal EPDV with invasive disease recurred thrice. The first and second recurrence intervals were 62.1 (9-146) months and 22 (15-28) months, respectively. The rate of invasive disease was 23.7% (9/38) for primary EPDV and 25% (3/12) for recurrent ones. We determined that the presence of invasive disease was associated with a history of more than 10 years (p = 0.02) and inversely correlated with positive margins (p = 0.037), However, invasive disease had no statistical relations with age (p = 0.438), recurrence (p = 0.642), and lesion diameter (p = 0.08). CONCLUSIONS: EPDV with a history of more than 10 years was associated with invasive disease. Close and long-term follow-up are recommended to identify those who require further treatment.

Non-human-papillomavirus-related malignancies of the vulva: A clinicopathological study.

BACKGROUND: Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high-risk human papillomavirus (HPV) infection. METHODS: A search for non-HPV-related malignant tumors of the vulva was performed in the last 20 years (2000-2020) in the pathology database of a single tertiary institution. We aim to estimate the prevalence of non-HPV-related malignancies in our population, describe clinicopathological features of these tumors and investigate the expression of some potential therapeutic targets. RESULTS: A total of 71 patients were recovered; 26 patients (36%) had the diagnosis of extramammary Paget disease, 17 patients (24%) had basal cell carcinomas, 17 patients (24%) had primary melanomas, 10 patients (14%) had metastatic disease to the vulva and one patient (1%) had a primary dermatofibrosarcoma protuberans. Fifty-four percent of patients with extramammary Paget disease had a secondary malignancy and 12.5% had invasive disease. Programmed death-ligand 1 (PDL-1) was positive in seven out of nine primary melanomas and Her2/neu was overexpressed in six out of seven extramammary Paget disease. CONCLUSION: Non-HPV-related malignancies are important differential diagnoses in patient with vulvar lesions. Additional research is necessary to further understand these complex malignancies and potential new therapeutic targets.

Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up.

BACKGROUND: Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence. OBJECTIVE: To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis. MATERIALS AND METHODS: Literature review using PubMed search for articles related to EMPD. RESULTS: Extramammary Paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy. CONCLUSION: Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.

First-time versus recurrent penoscrotal extramammary Paget's disease: Clinicopathological characteristics and risk factors in 164 Chinese male patients.

BACKGROUND: Penoscrotal extramammary Paget's disease is a rare, slow-growing neoplasm with high frequency of local recurrence. AIMS: The aim of this study was to investigate the difference in clinicopathological characteristics between first-time and recurrent penoscrotal Paget's disease, and to discover the potential risk factors of recurrence. METHODS: Between January 2007 and February 2014, a total of 164 Chinese patients with biopsy-proven tramammary Paget's diseaseex in penis and scrotum underwent wide local resection in our institution. Among them, 142 patients with first-time disease and other 22 patients with recurrent disease were enrolled in this retrospective analysis. RESULTS: The median duration of symptoms was much shorter in recurrent disease than in first-timers (3 vs. 24 months, P < 0.001). Patients with recurrent disease tended to have lower lesion exudation rates (27.3% vs. 51.8%, P= 0.032). In addition, patients with distant stage were more likely to obtain recurrent disease compared with first-time disease (P = 0.005). Through immunohistochemical detection of extramammary Paget's specimen, we found that HER2/neu protein expression in the recurrent group was significantly higher than first-timers (P = 0.036). LIMITATIONS: In this study, the information on familial history of most patients was insufficient. Moreover, due to the lack of follow-up data of our included cases, we were unable to evaluate the prognosis after diagnosis of extramammary Paget's disease. CONCLUSION: Patients with penoscrotal Paget's disease, especially those with shorter duration of symptoms, exudation of lesions, distant-stage, Paget cells infiltrating into adnexa, and HER2/neu expression, should be followed up more carefully after surgery, as they were more likely to suffer recurrence.

High Risk of Proximal and Local Neoplasms in 2206 Patients With Anogenital Extramammary Paget's Disease.

BACKGROUND: Extramammary Paget's disease is an uncommon intraepidermal adenocarcinoma with poorly defined clinical implications. OBJECTIVE: The purpose of this research was to estimate the risk of second primary neoplasms in patients with extramammary Paget's disease. DESIGN: This was a retrospective analysis of the Surveillance, Epidemiology, and End Results Registry (1973-2014). SETTINGS: The study included population-based cancer registries from the United States. PATIENTS: Patients who were diagnosed with anogenital Paget's disease were included. MAIN OUTCOME MEASURES: Risk of second primary development was measured. RESULTS: We identified 108 patients with anal Paget's disease, 421 patients with male genital (scrotum or penis) Paget's, and 1677 patients with female genital (vagina or vulva) Paget's. Median follow-up time was 5.9 years. The risk of developing colorectal adenocarcinoma was 18.5% for patients with anal Paget's disease. Eighty percent of colorectal adenocarcinoma diagnoses were synchronous (within 2 mo) to anal Paget's diagnoses, whereas metachronous tumors occurred at a median time of 2.4 years. Of patients with anal Paget's disease, 8.3% developed an anal adenocarcinoma or nonsmall cell cancer. In male patients with genital Paget's, the risk of proximal genitourinary malignancy was 9.7%, scrotal or testicular adenocarcinoma was 0.4%, and penile or scrotal squamous carcinoma was 1.7%. In female patients with genital Paget's, the risk of proximal genitourinary malignancy was 3.0%, vaginal or vulvar adenocarcinoma was 1.4%, and vaginal or vulvar squamous neoplasm was 1.0%. Five-year overall survival was 59.7%, 73.5%, and 80.7% in patients with anal, male genital, and female genital Paget's (p < 0.001). LIMITATIONS: The registry did not record surveillance schedule, provider specialty, or nonprocedural therapies for extramammary Paget's disease. CONCLUSIONS: In the largest published cohort of patients with extramammary Paget's disease, patients with anal Paget's had a much higher risk of both proximal and local neoplasms as compared with patients with genital Paget's. Patients with anal Paget's also experienced worse survival as compared with those with purely genital Paget's. See Video Abstract at http://links.lww.com/DCR/B20. ALTO RIESGO DE NEOPLASIAS PROXIMALES Y LOCALES EN 2206 PACIENTES CON ENFERMEDAD DE PAGET EXTRAMAMARIA ANOGENITAL:: La enfermedad de Paget extramamaria es un adenocarcinoma intraepidérmico poco frecuente con implicaciones clínicas poco definidas.Estimar el riesgo de segundas neoplasias primarias en pacientes con enfermedad de Paget extramamaria.Análisis retrospectivo del Registro de Vigilancia, Epidemiología y Resultados Finales (1973-2014).Registros de base poblacional en cáncer de los Estados Unidos.Pacientes que fueron diagnosticados con enfermedad de Paget anogenital.Riesgo de desarrollo un cáncer primario adicional.Se identificaron 108 pacientes con Paget anal, 421 pacientes con Paget genital masculino (escroto o pene) y 1677 pacientes con Paget genital femenino (vagina o vulva). Tiempo mediano de seguimiento fue de 5,9 años. El riesgo de desarrollar adenocarcinoma colorrectal fue del 18,5% para los pacientes con Paget anal. El ochenta por ciento de los diagnósticos de adenocarcinoma colorrectal fueron sincrónicos (dentro de los 2 meses) a los diagnósticos de Paget anal, mientras que los tumores metacrónicos ocurrieron en un tiempo promedio de 2,4 años. De los pacientes con Paget anal, el 8.3% desarrolló un adenocarcinoma anal o cáncer de células no pequeñas. En los pacientes masculinos con Paget genital, el riesgo de malignidad genitourinaria proximal fue del 9,7%, el adenocarcinoma escrotal o testicular fue del 0,4% y el carcinoma escamoso del pene o escroto fue del 1,7%. En pacientes femeninas con Paget genital, el riesgo de malignidad genitourinaria proximal fue de 3.0%, el adenocarcinoma vaginal o vulvar fue de 1.4% y la neoplasia escamosa vaginal o vulvar fue de 1.0%. La supervivencia general a cinco años fue del 59.7%, 73.5% y 80.7% en pacientes con anal, genital masculino y genital femenino, respectivamente (p <0.001).El registro no señalo el cronograma de vigilancia, la especialidad del proveedor o las terapias sin procedimiento para la enfermedad de Paget extramamaria.En la cohorte más grande publicada de pacientes con enfermedad de Paget extramamaria, los pacientes con Paget anal demostraron un riesgo mucho mayor de neoplasias proximales y locales en comparación con los pacientes con Paget genital. Los pacientes con Paget anal además demostraron una peor supervivencia en comparación con aquellos con Paget aislada genital. Vea el Resumen del Video en http://links.lww.com/DCR/B20.

Characteristics of skin cancers among adult patients in an urban Malaysian population.

There has been a rising incidence of skin cancers among Asians in recent years. We present a retrospective analysis of 106 skin cancers and analysed the demography, clinical subtypes of skin cancers and surgical techniques used for skin cancer treatment. In our population, skin cancers were most frequently basal cell carcinomas and diagnosed among ethnic Chinese patients.

Extramammary Paget disease.

In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease.

Nation-wide survey of advanced non-melanoma skin cancers treated at dermatology departments in Japan.

BACKGROUND: There are limited treatment options for advanced non-melanoma skin cancers (NMSCs). To overcome this issue, we need to conduct clinical studies, however, there is a lack of information on how many patients with advanced NMSCs are treated annually in Japan. OBJECTIVE: To investigate the actual number of advanced NMSC patients in Japan. METHODS: A questionnaire survey was sent to 668 institutes to educe information on: 1) the numbers of patients with squamous cell carcinoma (SCC), extramammary Paget disease (EMPD), other skin origin carcinomas, and cutaneous angiosarcoma (CAS) admitted in 2016 and 2017; 2) the preferred first- and second-line chemotherapies; and 3) the anticipated for future development. RESULTS: Questionnaires were returned from 383 (57.3%) institutes. They reported a total of 1765 patients over the 2 years. The annual number patients with SCC, EMPD, other skin carcinomas, and CAS was 323.5, 192.5, 126, and 240.5, respectively. We estimated the annual number of patients for all 668 institutes to be 1255.6. Current first- and second-line treatment for NMSCs were chemotherapy regimens, but immune checkpoint inhibitors were the most anticipated new drugs for SCC and CAS, while chemotherapy was still the most anticipated treatment for EMPD. CONCLUSION: Considering that during 2017, the number of deaths in Japan due to NMSC was reported to be 948, our estimated annual number of patients with NMSCs, 1255.6 seems to be an accurate estimation. As most of the treatment options for advanced NMSCs are outdated, the results of this study should be used to propose clinical studies.

Survival analysis of patients with invasive extramammary Paget disease: implications of anatomic sites.

BACKGROUND: Extramammary Paget disease (EMPD) is a rare malignant dermatosis with poorly defined outcomes. We investigated clinical characteristics of invasive EMPD at different anatomic sites and by subject demographics to determine prognostic factors for overall survival (OS). METHODS: All patient data were collected from the Surveillance, Epidemiology, and End Results (SEER) program, 1973-2013, of the U.S. National Cancer Institute. Patients with invasive EMPD of skin, vulva/labia, vagina, scrotum/penis, or other sites were included. After excluding patients with unknown radiation status, data of 2001 patients were analyzed. Primary endpoint was EMPD mortality by anatomic sites. Independent variables included patients' demographic data, concurrent malignancy (ie, non-EMPD related cancers), tumor size, distant metastasis, and surgery and/or radiation or not. RESULTS: Multivariate regression analysis showed that mortality was significantly higher in patients with vaginal EMPD than in patients with vulvar/labial EMPD (adjusted hazard ratio [aHR] = 3.26, p < 0.001). Patients with distant metastasis had higher mortality than those without (aHR = 3.36, p < 0.001). Patients who received surgery had significantly lower mortality than those who did not receive surgery (aHR = 0.77, p = 0.030), and those treated with radiation had significantly higher mortality than those who did not receive radiation (aHR = 1.60, p = 0.002). Older age was associated with significantly increased mortality (aHR = 1.09, p < 0.001), and mortality was significantly higher in males than in females (aHR = 1.42, p = 0.008). CONCLUSIONS: In conclusion, among EMPD patients, mortality is higher in patients with vaginal EMPD than in those with vulvar/labial EMPD and higher in those who are older, those with concurrent malignancy or distant metastasis. Mortality is also higher in males than in females. Surgery is a protective factor and radiation is a risk factor for OS. Greater understanding of EMPD clinical characteristics, and considering EMPD in differential diagnosis of chronic genital and perianal dermatoses may provide support for early EMPD diagnosis and definitive surgical treatment.

Results of second-stage screening for skin cancers in Oita Prefecture, Japan.

We performed skin cancer screenings for 2 or 3 days annually from 2006 through 2013 in Oita Prefecture, Japan. Screening of approximately 3000 people in total allowed us to identify and treat several skin cancers, including five cases of malignant melanoma, four of squamous cell carcinoma, 16 of basal cell carcinoma, 11 of Bowen's disease, 17 of actinic keratosis, one of extramammary Paget's disease and one of metastatic breast carcinoma. The sensitivity and specificity for the category defined by an identified lesion associated with risk of cancer and requiring further examination (category C) were 92.7% and 95%, respectively. We cannot estimate the outcome of our skin cancer screenings in terms of cancer mortality because of the small number of subjects examined and the brief follow-up period. However, we did estimate the effectiveness of these screenings in terms of stages or sizes of cancerous lesions. The relative numbers of subjects with malignant melanoma at various clinical stages, identified during skin cancer screenings and during a routine visit to our hospital, were significantly different. We also compared, statistically, the sizes of lesions in Bowen's disease that were found during cancer screenings and during a direct visit to our hospital. The former lesions were smaller than the latter. Our data suggest the benefits of our skin cancer screenings and the importance of campaigns and education to encourage people to visit dermatologists for the detection of skin cancers at an early stage.