Prognosis

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A territory wide follow-up of primary and secondary extramammary Paget disease of two decades – effects of local disease on survival

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A territory wide follow-up of primary and secondary extramammary Paget disease of two decades – effects of local disease on survival

Extramammary Paget disease (EMPD) is an uncommon malignant cutaneous neoplasm that are divided into primary and secondary forms. In this multicenter study, histologically proven cases of primary and secondary EMPD were reviewed for clinical outcomes with subgroup analysis for secondary EMPD.Secondary EMPD is associated with poorer outcomes. Margin involvement is strong and independent indicator of shorter OS and DSS, including secondary EMPD. Resectability is a strong predictor of favorable outcome and excision with clear margins should be attempted when surgically feasible.

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Risk of mortality from visceral malignancies in patients with extramammary Paget’s disease: a Surveillance, Epidemiology, and End Results (SEER) program-based analysis

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Risk of mortality from visceral malignancies in patients with extramammary Paget’s disease: a Surveillance, Epidemiology, and End Results (SEER) program-based analysis

The review summarizes extramammary Paget's disease (EMPD), emphasizing the increased risk of internal malignancies and the need for thorough cancer screening. Analyzing data from 3,723 EMPD patients in the SEER program, researchers found a significant rise in mortality from breast (SMR 2.62) and colorectal cancers (SMR 1.83), with 272 cancer-related deaths overall (SMR 2.08). Notably, bladder, prostate, and gastric cancers did not show increased mortality risk. The findings support current guidelines for age-appropriate colonoscopy and mammography in EMPD patients while questioning the necessity for universal screening for bladder and prostate cancers. Limitations include potential misclassification and unaccounted lifestyle or socioeconomic factors.

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Independent Risk Factors for Postoperative Recurrence of Patients with Primary Extramammary Paget’s Disease: A Retrospective Analysis

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Independent Risk Factors for Postoperative Recurrence of Patients with Primary Extramammary Paget’s Disease: A Retrospective Analysis

Primary extramammary Paget’s disease (EMPD) is a rare type of intraepithelial adenocarcinoma that shares histological features with Paget's disease but affects areas rich in apocrine glands outside the breast. It predominantly manifests in regions like the vulva, perianal, scrotum, and penile areas, with symptoms such as pruritus, erythema, and occasionally nodules or exudation. Unlike mammary Paget’s disease, primary EMPD affects both sexes, with a higher prevalence in Asian men and white women. The disease is rare, with an incidence of approximately 0.4-0.6 per million person-years in Europe and China. Despite its slow growth and nonspecific symptoms, primary EMPD carries significant morbidity due to high recurrence rates following surgical resection, prompting studies to identify prognostic factors like ill-defined tumor borders, exudation, and nodules.

A recent retrospective study from the First Affiliated Hospital of Chongqing Medical University aimed to analyze factors influencing postoperative recurrence of primary EMPD. The study enrolled 40 Chinese patients diagnosed between 2009 and 2019, predominantly male (77.5%), with a median age of 75.52 years. Clinical characteristics such as ill-defined tumor borders, presence of exudation, and nodules on the lesions were identified as significant risk factors for recurrence in multivariate analysis. Despite advancements in surgical techniques like wide local excision and Mohs micrographic surgery, the study highlighted challenges in early diagnosis due to misdiagnosis and delayed treatment, often mistaken for common dermatological conditions. The findings underscored the importance of vigilant clinical assessment and the need for tailored surgical approaches to improve outcomes in primary EMPD. This study contributes insights into managing primary EMPD by identifying specific clinical features associated with recurrence risk. Further research with larger cohorts and longer follow-up periods is necessary to validate these findings and refine treatment strategies for this challenging disease.

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National incidence and survival of extramammary Paget disease 2013–2020 in England: reporting a total of 740 cases

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National incidence and survival of extramammary Paget disease 2013–2020 in England: reporting a total of 740 cases

Extramammary Paget disease (EMPD) is a rare cancer primarily affecting genital and perianal areas, first described in 1889. Diagnosis can be delayed due to its subtle symptoms, but it's crucial as it often signals underlying malignancies, most commonly urothelial or anorectal. Recent epidemiological data from England suggest a fluctuating annual incidence around 0.17 per 100,000 person-years, with a noticeable decrease during the COVID-19 pandemic, highlighting its impact on diagnostic and healthcare services.

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Incidence and Survival of Extramammary Paget’s Disease from the Surveillance, Epidemiology, and End Results (SEER) Database

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Incidence and Survival of Extramammary Paget’s Disease from the Surveillance, Epidemiology, and End Results (SEER) Database

Extramammary Paget’s Disease (EMPD) is a rare intraepithelial neoplasm predominantly affecting the anogenital regions of older, female, and Asian patients. It often progresses insidiously, leading to diagnostic delays spanning years. This study, conducted through a retrospective review of EMPD patients from 22 SEER registries between 2004 and 2020, aimed to analyze contemporary trends in incidence and survival across sexes. The data included various demographics and clinical factors, and age-adjusted incidence rates were calculated over a 20-year period. Survival estimates were assessed using Kaplan-Meier curves, while Cox proportional hazards models were utilized to identify factors associated with all-cause mortality. The study included 3608 patients, with a higher incidence in Asian patients, who had a rate double that of white patients. Notably, the incidence increase was significant only among white patients. Females had a better ten-year survival rate (63.0%) compared to males (53.4%). Multivariable analysis revealed that older age, advanced disease stage, and treatment delays were linked to poorer overall survival, while primary surgical treatment significantly reduced mortality risk compared to no cancer-directed treatment. The study highlights the rising incidence of EMPD and emphasizes the importance of early, complete surgical resection in improving long-term survival outcomes.

Anatomic Subtype Differences in Extramammary Paget Disease

Anatomic Subtype Differences in Extramammary Paget Disease

How do disease presentation and treatment in extramammary Paget disease (EMPD) differ by anatomic subtype? This meta-analysis of 135 studies found that, despite typically being intraepidermal, vulvar disease was more likely treated with radical surgery. Nearly one-third of vulvar cases recurred, predominantly locally in the skin and mucosa and one-third of perianal EMPD cases recurred; however, one-third of those recurrences presented as regional or distant metastasis and penoscrotal EMPD was least likely to recur.

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Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients

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Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients

Extramammary Paget's disease (EMPD) is rare. There are no standard treatments due to its rarity and few clinical trials. The 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. Due 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.

Nomograms for predicting cancer-specific and overall survival in patients with invasive extramammary Paget’s disease

Nomograms for predicting cancer-specific and overall survival in patients with invasive extramammary Paget’s disease

Invasive extramammary Paget’s disease (iEMPD) is a rare type of cutaneous malignancy with a heterogeneous prognosis. The purpose of this study is to identify the prognostic factors associated with cancer-specific and overall survival rates in iEMPD and to develop accurate risk stratification models to guide the design of individualized treatment regimens. The nomograms can be a reliable tool for treatment design and prognostic evaluation of iEMPD.

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Prognostic value of treatment options for extramammary Paget’s disease: a SEER database analysi

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Prognostic value of treatment options for extramammary Paget’s disease: a SEER database analysi

Extramammary Paget’s disease (EMPD) is a common subtype of Paget’s disease. Still, there are lacking reports concerning its clinical features, treatment options, and prognosis. EMPD had distinct clinical features from MPD. Age, gender, chemotherapy, tumor grade and stage are independent prognostic factors for EMPD. While surgery’s protective role was supported, radiotherapy and chemotherapy could be unfavorable treatments concerning EMPD prognosis.

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The Outcome of Chemotherapy for Metastatic Extramammary Paget’s Disease

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The Outcome of Chemotherapy for Metastatic Extramammary Paget’s Disease

The efficacy and survival impact of conventional chemotherapies for metastatic extra- mammary Paget’s disease (EMPD) have not been fully elucidated. This study examined the long- term outcome of chemotherapy for this indication. We conducted a retrospective review of 21 pa- tients with distant metastatic EMPD (14 patients treated with chemotherapy and 7 patients treated without chemotherapy). The response rate of chemotherapy and patient survival were statistically analyzed.

Prognostic Factors of Extramammary Paget’s Disease

Prognostic Factors of Extramammary Paget’s Disease

Most patients with extramammary Paget’s disease (EMPD) show a good prognosis; however, some patients develop fatal metastases. Early detection is important for improving prognosis, due to the difficulties associated with the treatment of distant EMPD metastases. Several studies have emphasized the importance of the invasion level of the primary lesion for predicting the presence of metastasis, and deeper invasion or increased thickness is correlated with poorer prognosis. Vascular tumor invasion of the primary lesion can also predict the risk of metastasis. Lymph node metastasis is a strong indicator for poor prognosis, and the number of lymph node metastases affects patient outcome, in that there is a significant difference in survival between patients with zero or one lymph node metastasis and those with more than two lymph node metastases. Serum markers may be able to predict the presence of systemic metastases, and carcinoembryonic antigen and cytokeratin 19 fragment 21-1 reflect disease progression and may be clinically valuable. Although several genetic alterations have been determined for EMPD, factors determining prognosis should be further explored.

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Survival analysis of patients with invasive extramammary Paget disease -- Implications of anatomic sites

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Survival analysis of patients with invasive extramammary Paget disease -- Implications of anatomic sites

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). 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. 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). 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.

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Multicentric Primary Extramammary Paget Disease: A Toker Cell Disorder?

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Multicentric Primary Extramammary Paget Disease: A Toker Cell Disorder?

Toker cells are epithelial clear cells found in the areolar and nipple areas of the breast, vulvar region, and other apocrine gland–bearing areas of the skin. Toker cells have been implicated in the pathogenesis of clear cell papulosis, cutaneous hamartoma with pagetoid cells, and rare cases of primary extramammary Paget disease (EMPD) but not in secondary EMPD with underlying adenocarcinoma. The pathogenesis of primary EMPD is not well defined. We report a case of multicentric primary EMPD with evidence of Toker cell proliferation and nonaggressive biologic behavior in a 63-year-old white man. A detailed description of the morphologic and biologic features of Toker cells and their possible carcinogenetic links also are discussed. Based on the observation and follow-up of our patient, we hypothesize that multicentric primary EMPD starts with Toker cell hyperplasia and can potentially evolve to carcinoma in the genital region.

Extramammary Paget disease (EMPD), which was first described by Crocker1 in a patient with erythematous patches on the penis and scrotum, is morphologically identical to mammary Paget disease (MPD) of the nipple. The principal difference between EMPD and MPD is anatomic location.

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Prognosis and Management of Extramammary Paget’s Disease and the Association with Secondary Malignancies

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Prognosis and Management of Extramammary Paget’s Disease and the Association with Secondary Malignancies

Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p 􏰀 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population.

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