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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.

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Efficacy of chemotherapies for unresectable extramammary Paget disease: a single-centre retrospective study

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Efficacy of chemotherapies for unresectable extramammary Paget disease: a single-centre retrospective study

Extramammary Paget disease (EMPD) is a cutaneous neoplasm that can metastasise to the lymph nodes and distant organs, resulting in a poor prognosis. For unresectable distant metastases of EMPD, no consensus has been reached regarding the optimal chemotherapy owing to the lack of data. Further investigations with prospective analysis are required to confirm these findings.

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Five- and 10-year survival in extramammary Paget's disease: A focus on wide local excision

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Five- and 10-year survival in extramammary Paget's disease: A focus on wide local excision

This study aimed to analyze extramammary Paget's disease (EMPD)-specific survival, overall survival, and recurrence rate (RR) in patients with EMPD in South Korea, with a focus on wide local excision. Based on the survival and RRs obtained, surgical treatment of EMPD with wide local excision provides fair curative resection.

Extramammary Paget's disease involving the axilla: case series and literature review

Extramammary Paget's disease involving the axilla: case series and literature review

Axillary extramammary Paget's disease (EMPD) is a rare condition with only a few cases reported in the literature. We performed a retrospective review and identified 16 cases of EMPD with axillary involvement. None of the patients developed recurrence or metastasis after surgery at a mean follow-up of 63.6 months. Axillary EMPD shares similar clinicopathological features with typical EMPD.

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TRPS1 expression is sensitive and specific for primary extramammary Paget disease

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TRPS1 expression is sensitive and specific for primary extramammary Paget disease

The most frequently utilized biomarkers for confirming the diagnosis of EMPD include CK7 and GATA3; however, these biomarkers lack specificity. The purpose of this study was to evaluate TRPS1, a newly described breast biomarker, in pagetoid neoplasms of the vulva, scrotum and anorectum. These results demonstrate that TRPS1 is a sensitive and specific biomarker for EMPD, and may be especially useful for excluding secondary involvement of the vulva by urothelial and anorectal carcinomas.

Analysis of perianal lesions associated with anal canal adenocarcinoma and perianal primary extramammary Paget disease

Analysis of perianal lesions associated with anal canal adenocarcinoma and perianal primary extramammary Paget disease

Secondary extramammary Paget disease (s-EMPD) represents anal canal and rectal, bladder, and gynecological cancers, which horizontally extend within the epidermis of the anal and vulvar skin. It is necessary to distinguish this condition from primary extramammary Paget disease (p-EMPD), which occurs primarily in genital and perianal areas. This study aimed to investigate the clinical and histopathological features of these two conditions in the perianal skin and to identify useful features for differentiation. We retrospectively analyzed 16 patients who visited Shinshu University Hospital from 2009 to 2022 and presented with perianal skin lesions and suspected EMPD. Six patients had p-EMPD and 10 had s-EMPD derived from anal canal adenocarcinoma. Regarding clinical features, nine of 10 (90%) of the s-EMPD cases had symmetric skin lesions, whereas all of the p-EMPD cases had asymmetrical lesions (p = 0.0004). Furthermore, assessment of symmetry around the anus showed that s-EMPD had a significantly smaller coefficient of variation than p-EMPD (0.35 and 0.62, respectively; p = 0.048), suggesting that s-EMPD was more symmetric around the anus. The frequency of raised lesions, such as foci or nodules, was nine of 10 (90%) for s-EMPD and one of six (16%) for p-EMPD (p = 0.003). Well-defined tumor borders on the lateral margins were identified in s-EMPD (5/10, 50%); however, they were not identified in p-EMPD (0/6, 0%). The borders tended to be clearer in s-EMPD; however, the difference was not significant (p = 0.078). Based on these findings, we recommend consideration of s-EMPD when anal skin lesions are symmetrical, well-defined, or raised.

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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