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Incidental finding of extramammary Paget's disease during active surveillance for early-stage prostate cancer in a prostate biopsy

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Incidental finding of extramammary Paget's disease during active surveillance for early-stage prostate cancer in a prostate biopsy

Skin tissue contamination within transcutaneous visceral organ biopsies is seldom found. We encountered a rare case of extramammary Paget's disease incidentally diagnosed by prostate biopsy during active surveillance for prostate cancer. Although the perianal region is a common site of extramammary Paget's disease, early-stage extramammary Paget's disease is often asymptomatic. Thus, during a transcutaneous biopsy, it is important to consider the appearance of the skin and the pathological features of migrating skin tissue.

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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.

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

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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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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Margin Assessment of Extramammary Paget's Disease Based on Harmonic Generation Microscopy with Deep Neural Networks

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Margin Assessment of Extramammary Paget's Disease Based on Harmonic Generation Microscopy with Deep Neural Networks

Surgical borders of extramammary Paget's disease (EMPD) are difficult to be identified via its clinical appearance. In this study, we propose a new diagnostic technique which combines nonlinear harmonic generation microscopy (HGM) with the deep learning method to instantaneously determine whether the imaged 3D stack is malignant EMPD or surrounding normal skin digitally. To demonstrate our proposal, in this study different locations of fresh EMPD surgical samples were 3D imaged starting from the surface up to a depth of 180 m using stain-free HGM. With the followed histopathological examination of the same sample, we mapped the gold-standard results to 3D HGM image stacks with labels for the training of the deep learning model. With only 2095 3D image stacks as training and validation data, the results of EMPD and normal skin tissue classification achieve 98.06% sensitivity, 93.18% specificity and 95.81% accuracy. This study supports our proposed 3D convolutional-neural-network-based technique with a high potential to assist physicians to quickly map the EMPD margins by providing noninvasive instant information regarding the imaged sub-millimeter site as malignant or surrounding normal with a high accuracy.

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Serum cytokeratin 19 fragment 21‐1 and carcinoembryonic antigen combination assay as a biomarker of tumour progression and treatment response in extramammary Paget's disease

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Serum cytokeratin 19 fragment 21‐1 and carcinoembryonic antigen combination assay as a biomarker of tumour progression and treatment response in extramammary Paget's disease

Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma affecting the genitals and axillary regions. As metastasis of these tumours is itself rare, solid disease management strategies have not been established. Serum carcinoembryonic antigen (CEA) and cytokeratin 19 fragment 21‐1 (CYFRA 21‐1) levels have been identified as candidate biomarkers for tumour progression in EMPD; however, neither the accuracy of, nor correlation between, these markers have been examined in EMPD patients.

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Serum cell‐free DNA levels are useful marker for extramammary Paget's disease

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Serum cell‐free DNA levels are useful marker for extramammary Paget's disease

Although carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA) are useful marker for extramammary Paget's disease (EMPD), the serum CEA and CYFRA levels are not elevated in most EMPD patients without metastasis. Therefore, further useful biomarkers are needed for the detection of EMPD including early lesions. Cell‐free DNA (cfDNA) has attracted attention as an indicator of clinical conditions in several cancers, and we studied the clinical implications of cfDNA for EMPD. The serum cfDNA levels were significantly elevated in EMPD patients with or without metastasis compared to those in healthy controls. And serum cfDNA was a better diagnostic marker for the presence of EMPD compared to serum CYFRA. Moreover, the post‐operative serum cfDNA levels were significantly lower than those from the pre‐operative samples, and the change in serum cfDNA levels reflected the clinical courses of EMPD patients treated with chemotherapy. Taken together, serum cfDNA levels may be useful marker for the diagnosis and disease progression in EMPD.

Mayo Clinic: Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease Study

Mayo Clinic: Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease Study

The Mayo Clinic (Rochester, MN, USA) is currently recruiting women with vulvar Extramammary Paget's Disease for an EMPD study. This includes EMPD of the vulva, vagina or perianal EMPD. The 25-year study will allow physicians to better understand EMPD. The researchers also want to learn more about the microorganisms (microbiome) that live on or near the areas of skin affected by EMPD, in order to better understand this condition. Tumor tissues will be obtained in order to perform tumor molecular profiling to identify targetable somatic mutations in EMPD. This EMPD study is for women only. More information on the Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease study can be found here. 

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Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease

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Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease

To identify the rates of associated and occult cancers in patients with extramammary Paget disease (EMPD) discovered using cancer screening methods at a tertiary medical center; to propose evidence-based cancer screening guidelines at the time of diagnosis of EMPD; and to clarify terminology associating EMPD with underlying malignancies.

All patients diagnosed with EMPD should undergo cancer screening. At minimum, evaluation should include age-appropriate screening and the addition of urine cytology, mammography, and prostate-specific antigen blood test—if not already performed—may be of particular use. An algorithm for evaluation of patients with newly diagnosed EMPD is proposed.

Serum cytokeratin 19 fragment 21-1 is a useful tumor marker for the assessment of extramammary Paget's disease.

Serum cytokeratin 19 fragment 21-1 is a useful tumor marker for the assessment of extramammary Paget's disease.

Cytokeratin 19 fragment 21-1 (CYFRA 21-1) has been used as a tumor marker for several malignancies. However, to date, no studies have assessed whether CYFRA 21-1 could be a useful marker for extramammary Paget's disease (EMPD). The present study aimed to evaluate the significance of CYFRA 21-1 as a serum tumor marker for EMPD progression. Concentrations of serum CYFRA 21-1 and carcinoembryonic antigen (CEA) in 13 cases of EMPD were measured prior to undergoing treatment at Sapporo Medical University Hospital from January 2014 to May 2016. Four of the 13 patients had lymph node metastases at diagnosis, but none had distant metastases. Immunohistochemistry indicated that all 13 primary tumors and four metastatic tumors in lymph nodes were positive for cytokeratin 19. Although none of the 13 patients showed high serum CEA levels, six patients (46.2%) had elevated serum CYFRA 21-1. Furthermore, CYFRA 21-1 was reduced in association with post-treatment tumor reduction in all six patients. Among these six patients, four developed recurrence and metastasis during the follow-up period. CYFRA 21-1 was re-elevated in all four of these patients; however, serum CEA was elevated only in the patient with distant metastasis. These results suggest that CYFRA 21-1 is more sensitive compared with CEA, and can be useful as a tumor marker for evaluating tumor progression and treatment efficacy in patients with EMPD.

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