Docetaxel treatment for widely metastatic invasive vulvar extramammary Paget’s disease with multifocal bone metastasis

Docetaxel treatment for widely metastatic invasive vulvar extramammary Paget’s disease with multifocal bone metastasis

Invasive extramammary Paget’s disease (EMPD) of the vulva, first identified in 1901, typically affects Caucasians aged 50–65. Approximately 65% of EMPD cases occur in the vulva, representing 1–6% of vulvar cancers in the U.S., with an incidence of 0.36 per 100,000 person-years. Metastasis to lymph nodes worsens prognosis, and 44% of Japanese patients with regional lymph node metastasis develop distant metastasis. Due to the disease's rarity, high-quality interventional or outcomes data are scarce, and randomized clinical trials are unlikely. Treatment for metastatic EMPD is based on retrospective case reports or small series, with varying regimens including monotherapy (docetaxel, cisplatin, 5-FU) and multi-agent chemotherapy (5-FU/cisplatin, cisplatin/epirubicin/paclitaxel). Specific treatment details and efficacy for metastatic vulvar EMPD are lacking. This report highlights a case where monthly low-dose docetaxel achieved a highly effective, durable response with minimal toxicity.

Dermoscopy as a Diagnostic and Monitoring Tool for Recurrent Extramammary Paget's Disease

Dermoscopy as a Diagnostic and Monitoring Tool for Recurrent Extramammary Paget's Disease

Extramammary Paget's disease (EMPD) is a rare skin cancer occurring in areas with abundant apocrine glands. Dermoscopy has been used to monitor treatment outcomes and post-treatment follow-up in skin cancers such as Bowen's disease, basal cell carcinoma, and squamous cell carcinoma. Sampling bias from incisional biopsies could be prevented, as the entire skin lesion could be examined. Therefore, dermoscopy is a useful tool to assess and detect highly recurrent tumors such as EMPD, thus improving the clinical prognosis of the patient.

A clinicopathological study of perianal Paget disease: A single center-based cohort study and literature review

A clinicopathological study of perianal Paget disease: A single center-based cohort study and literature review

Perianal Paget disease (PPD), an uncommon extramammary Paget disease, is characterized by intraepidermal pagetoid spread of atypical Paget cells in the perianal skin. PPDs can be primary or secondary. We report the clinicopathological features of 8 cases of PPD in Taiwanese and first describe differential CK7 expression in the epidermal and dermal tumor cells in 2 cases of secondary PPDs, which may provide a clue to the diagnosis of secondary PPD. Since an underlying anorectal carcinoma in PPDs may be undetectable by colonoscopy, it is essential to consider anoscopy and/or anal canal mucosal biopsy to search for an occult anorectal carcinoma.

Scrotal Extramammary Paget’s Disease in an Elderly Caucasian Male

Scrotal Extramammary Paget’s Disease in an Elderly Caucasian Male

Extramammary Paget’s disease (EMPD) is a rare dermatologic malignancy affecting regions with a dense population of apocrine glands within the intraepithelial tissue, including the vulva, perineum, axilla, scrotum, and penile regions. Clinical presentation varies from being asymptomatic to burning, painful, and pruritic lesions. As a result, it could be misdiagnosed for other dermatologic diseases. Our case report discloses a patient with an erythematous pruritic lesion that was initially treated with topical antifungal therapy. After failed treatment, a biopsy revealed EMPD of the scrotum. With no standard guidelines in the treatment of EMPD, there are different treatment modalities for the disease. Mohs micrographic surgery currently is the preferred treatment modality presenting with the lowest rates of recurrence. With early diagnosis and treatment, the five-year survival rate for patients with primary EMPD is 87%. Therefore, there should be a high level of clinical suspicion for EMPD in patients presenting with pruritic lesions in areas with apocrine glands that have failed initial medical treatment.

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Extramammary Paget's disease: Updates in the workup and management

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Extramammary Paget's disease: Updates in the workup and management

Extramammary Paget's disease (EMPD) is a rare malignancy primarily affecting apocrine gland-bearing areas such as the vulva, scrotum, and penis, with limited treatment data due to its infrequency. This review, based on literature up to February 2022, outlines a contemporary approach to diagnosing and managing EMPD. Typically presenting as an indolent carcinoma in situ, the cornerstone of treatment is prompt and accurate diagnosis followed by wide local or Mohs micrographic surgical excision, with careful management of margin status and consideration of lymphadenectomy for regionally positive disease. While conventional chemotherapies serve as alternatives for patients with distant metastases, their efficacy can be suboptimal, and consensus on adjuvant or systemic therapies is lacking. Recent advances in understanding the molecular pathogenesis and genomics of EMPD may pave the way for targeted treatments, underscoring the importance of specialized care, vigilant follow-up, and global collaborative efforts to design robust clinical trials for this orphan disease.

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Efficacy of Abemaciclib in the Management of Refractory Metastatic Extramammary Paget’s Disease

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Published systemic therapy options for metastatic extramammary Paget's disease have largely been anecdotal due to the rarity of this disease, which has precluded the ability to conduct clinical trials. We describe the favorable response of a 72-year-old man with extramammary Paget's disease, whose disease has been controlled with the CDK4/6 inhibitor, abemaciclib. The rationale behind the selection of this therapy is discussed.

Postoperative radiation therapy improves prognoses in extramammary Paget's disease presenting with multiple lymph node metastases

Postoperative radiation therapy improves prognoses in extramammary Paget's disease presenting with multiple lymph node metastases

Invasive extramammary Paget's disease may cause lymph node and distant metastases. Complete lymph node dissection is generally performed for extramammary Paget's disease presenting with lymph node metastases.

Thus, survival was significantly prolonged with postoperative radiotherapy. Additional postoperative radiotherapy may substantially improve the prognoses of patients with extramammary Paget's disease and ≥3 lymph node metastases, and undergoing curative surgery.

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years.

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Integrative bioinformatics approaches to map key biological markers and therapeutic drugs in Extramammary Paget’s disease of the scrotum

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Extramammary Paget’s disease (EMPD) is an intra-epidermal adenocarcinoma. Till now, the mechanisms underlying the pathogenesis of scrotal EMPD is poorly known. This present study aims to explore the knowledge of molecular mechanism of scrotal EMPD by identifying the hub genes and candidate drugs using integrated bioinformatics approaches. Firstly, the microarray datasets (GSE117285) were downloaded from the GEO database and then analyzed using GEO2R in order to obtain differentially expressed genes (DEGs). Moreover, hub genes were identified on the basis of their degree of connectivity using Cytohubba plugin of cytoscape tool. Finally, GEPIA and DGIdb were used for the survival analysis and selection of therapeutic candidates, respectively. A total of 786 DEGs were identified, of which 10 genes were considered as hub genes on the basis of the highest degree of connectivity. After the survival analysis of ten hub genes, a total of 5 genes were found to be altered in EMPD patients. Furthermore, 14 drugs of CHEK1, CCNA2, and CDK1 were found to have therapeutic potential against EMPD. This study updates the information and yields a new perspective in the context of understanding the pathogenesis of EMPD. In future, hub genes and candidate drugs might be capable of improving the personalized detection and therapies for EMPD.

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GATA-binding protein 3 and gross cystic disease fluid protein 15 as a potential diagnostic marker for extramammary Paget's disease

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The aim of this study was to evaluate the expression of GCDFP15 and GATA-binding protein 3 (GATA-3) in extramammary Paget's disease (EMPD) skin and serum samples and to assess their availability as tumor markers for the diagnosis and assessment of disease severity in primary EMPD. By immunohistochemistry, the expression of GCDFP15 and GATA3 was examined in skin specimens. There was no correlation between serum levels of GCDFP15 or GATA-3 and the disease stage. Our results indicate that GCDFP15 and GATA-3 are useful for the diag- nosis of primary EMPD, but not for monitoring disease progression, and suggest that GATA-3 is a more reliable marker than GCDFP15 for the diagnosis of primary EMPD.

A Positive Dermcidin Expression Is an Unfavorable Prognostic Marker for Extramammary Paget’s Disease

A Positive Dermcidin Expression Is an Unfavorable Prognostic Marker for Extramammary Paget’s Disease

Extramammary Paget’s disease is recognized as an apocrine-origin cutaneous tumor and is localized in the intraepithelial skin lesion. However, its advanced form is intractable, and there is currently no therapeutic option with a satisfactory level of clinical outcome. Therefore, it is of great importance to identify a potential biomarker to estimate tumor advancement in extramammary Paget’s disease. Dermcidin is an antimicrobial peptide derived from the eccrine gland and is identified as a biomarker in various malignancies. To investigate the potential of dermcidin in extramammary Paget’s disease, we investigated dermcidin expression in tumors using the immunostaining technique. Although previous studies have reported that extramammary Paget’s disease has no positive staining against dermcidin, 14 out of 60 patients showed positive staining of dermcidin in our study. To clarify the characteristics of positive dermcidin in extramammary Paget’s disease, we investigated the clinical characteristics of positive dermcidin extramammary Paget’s disease patients. Positive dermcidin patients showed a significantly high frequency of lymph node metastasis. We next investigated the impact of positive dermcidin on overall survival. Univariate analysis identified that positive dermcidin showed a significantly increased hazard ratio in overall survival, suggesting that dermcidin might be a prognostic factor for extramammary Paget’s disease.

A Positive Dermcidin Expression Is an Unfavorable Prognostic Marker for Extramammary Paget’s Disease

A Positive Dermcidin Expression Is an Unfavorable Prognostic Marker for Extramammary Paget’s Disease

Extramammary Paget’s disease is recognized as an apocrine-origin cutaneous tumor and is localized in the intraepithelial skin lesion. However, its advanced form is intractable, and there is currently no therapeutic option with a satisfactory level of clinical outcome. Therefore, it is of great importance to identify a potential biomarker to estimate tumor advancement in extramammary Paget’s disease. Dermcidin is an antimicrobial peptide derived from the eccrine gland and is identified as a biomarker in various malignancies. To investigate the potential of dermcidin in extramammary Paget’s disease, we investigated dermcidin expression in tumors using the immunostaining technique. Although previous studies have reported that extramammary Paget’s disease has no positive staining against dermcidin, 14 out of 60 patients showed positive staining of dermcidin in our study. To clarify the characteristics of positive dermcidin in extramammary Paget’s disease, we investigated the clinical characteristics of positive dermcidin extramammary Paget’s disease patients. Positive dermcidin patients showed a significantly high frequency of lymph node metastasis. We next investigated the impact of positive dermcidin on overall survival. Univariate analysis identified that positive dermcidin showed a significantly increased hazard ratio in overall survival, suggesting that dermcidin might be a prognostic factor for extramammary Paget’s disease.

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Well-demarcated erythematous plaque on the arm

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Well-demarcated erythematous plaque on the arm

A 67-year-old Caucasian man presented with an asymptomatic, well-defined, irregular shaped, erythematous scaly plaque on the left upper arm for five years. There was no cervical or axillary lymphadenopathy. A skin punch biopsy was performed from the plaque. Histopathological examination showed hyperkeratosis, acanthosis and atypical large pale cells with abundant vacuolated cytoplasm in the epidermis. There was no dermal invasion. Tumor cells were positive for cytokeratin 7, but negative for cytokeratin 20, P63 and Melan A by immunohistochemistry.

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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Overexpression of Janus kinase 2 protein in extramammary Paget’s disease

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Extramammary Paget’s disease is a rare malignant tumor of the skin that occurs primarily in the genitocrural region. Although the prognosis of extramammary Paget’s disease with distant metastasis is poor, an effective therapy has not been established. Because Janus kinase 2 has attracted attention as a therapeutic target in several cancers, we investigated the expression of the Janus kinase 2 protein and the relationship between its level of expression and clinical significance in 53 patients with extramammary Paget’s disease in our hospital. Immunohistochemistry showed that most extramammary Paget’s disease tissues were positive for Janus kinase 2 (50/53, 94.3%), and the immunostaining intensity of Janus kinase 2 was correlated with the degree of invasiveness, lymph node metastasis and distant metastasis. Based on these findings, Janus kinase 2 may be a promising therapeutic target in extramammary Paget’s disease.

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Expression of programmed death-ligand 1 and programmed death-1 in patients with extramammary paget's disease

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Expression of programmed death-ligand 1 and programmed death-1 in patients with extramammary paget's disease

Extramammary Paget's disease (EMPD) is a rare skin cancer and sometimes has fatal prognosis. For progressive cases, therapeutic options are limited. In recent years, treatment with an anti-programmed death-1 (PD-1) antibody has improved the prognosis of various malignancies. In addition, correlations between PD-ligand 1 (PD-L1) expression in tumor cells and favorable responses to anti-PD-1 therapy have been reported for several cancers. There have been a few case series of analysis of PD-L1 expression in patients with EMPD. The purpose of this study was to investigate the relationship between the EMPD and PD-L1/PD-1 expression in Japanese EMPD patients. We investigated 39 patients with EMPD by immunohistochemical staining of PD-L1 and PD-1. We counted the number of tumor cells that were positive for PD-L1 and the number of tumor-infiltrating mononuclear cells (TIMCs) that were positive for PD-L1 and PD-1. We also analyzed correlations between the expression of PD-L1 and PD-1 in EMPD and patients' characteristics. We found that none of the Paget's cells expressed PD-L1. All of the specimens contained TIMCs, and some of the TIMCs expressed PD-L1 and PD-1. However, there was no correlation between the expression of PD-L1/PD-1 in TIMCs and patients' characteristics. Although tumor cells did not express PD-L1 and the expression of PD-L1/PD-1 in TIMCs did not correlate with patients' characteristics, future clinical studies should be carried out to explore another immune escape pathway in EMPD.

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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.

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

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

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. We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. 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.

Definitive Radiotherapy for Penoscrotal Extramammary Paget’s Disease: A Case Report

Definitive Radiotherapy for Penoscrotal Extramammary Paget’s Disease: A Case Report

Penoscrotal extramammary Paget’s disease (EMPD) is a rare intraepithelial adenocarcinoma in older male patients, and no effective nonsurgical treatment strategies are currently available. The aim of this study was to report the usefulness of external radiotherapy (RT) for penoscrotal EMPD in an inoperable elderly patient. This report presents the treatment of an 89-year-old man with widespread penoscrotal EMPD. A multidisciplinary treatment team decided on radical RT. The patient received a radiation dose of 61.8 Gy in 30 fractions through electron and photon beams. His treatment tolerance was good, and no severe toxicity had been observed up the last follow-up. At 6.5 years after the RT, the patient showed no evidence of recurrence. Definitive RT resulted in excellent disease control and minimal toxicity; thus, it could be a promising nonsurgical therapeutic option for penoscrotal EMPD, even in extremely elderly individuals.

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Absence of microsatellite instability in extramammary Paget's disease

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Absence of microsatellite instability in extramammary Paget's disease

Deficiency of DNA mismatch repair (MMR) induces micro-satellite instability (MSI). Pembrolizumab, an antibody targeting PD‐1 (an immune checkpoint inhibitor), is more effective against MMR‐deficient tumours than against MMR‐proficient tumours. The status of MMR is a useful biomarker for predicting the effectiveness of pembrolizumab administration. Although the status of MMR has attracted attention in skin tumours, there are few reports on MSI in extramammary Paget's disease (EMPD). MSI testing showed the occurrence rates of MSI‐high (more than two markers are unstable), MSI‐low (one marker is unstable) and MSS (all markers are stable) tumour tissues were 0% (0/101), 1.0% (1/101) and 99.0% (100/101), respectively. The status of MMR may not be useful for the potential therapeutic application of pembrolizumab.

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