extramammary paget’s disease
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Author(s):  
Mariko Urano-Takaoka ◽  
Hayakazu Sumida ◽  
Takuya Miyagawa ◽  
Kentaro Awaji ◽  
Kojiro Nagai ◽  
...  

Extramammary Paget’s disease (EMPD) is a rare cutaneous adenocarcinoma with unfavorable prognosis once it becomes invasive. A tumor marker that reflects disease progression is required for adequate management of this disease. Cytokeratin 18 is highly expressed in many types of cancer and its soluble forms are detected by M30 (for caspase-cleaved form) and M65 (for both caspase-cleaved and intact forms) assays. Here, we report that tumor cells of EMPD in both lesional skin and lymph node metastasis are positive for CK18 immunohistochemically and the baseline serum M30 and M65 levels in metastatic EMPD patients are significantly higher than those in non-metastatic patients. In addition, serial serum M30 and M65 levels might reflect recurrence of EMPD and response to chemotherapy. These results suggest that serum CK18 levels may be a useful tumor marker for advanced EMPD.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Da-chao Zheng ◽  
Yan-ting Shen ◽  
Zi-wei Wei ◽  
Xiang Wan ◽  
Min-kai Xie ◽  
...  

Abstract Background Extramammary Paget’s disease (EMPD) is a rare malignant intraepidermal adenocarcinoma that is poorly understood. Regulatory long noncoding RNAs (lncRNAs) are characterized in many species and shown to be involved in processes such as development and pathologies, revealing a new layer of regulation in different diseases, especially in cancer studies. In the present study, we used high-throughput sequencing to reveal the lncRNA–mRNA interaction network in extramammary Paget’s disease. Methods High-throughput sequencing was used to identify differentially expressed lncRNA and mRNA profiles between EMPD patients and healthy controls. Then, a series of bioinformatics analyses were conducted to construct the lncRNA–mRNA interaction network, which was finally confirmed in vitro. Results Six pairs of EMPD tumor and normal skin samples were collected and sequenced to identify the differentially expressed lncRNA and mRNA profiles between EMPD and healthy controls. A total of 997 differentially expressed mRNAs and 785 differentially expressed lncRNAs were identified. The GO and KEGG analyses show that epidermal development and cell adhesion play important roles in EMPD. The results of the lncRNA–mRNA interaction network analysis suggested that NEAT1, PGAP1, FKBP5 and CDON were the pivotal nodes of the network and that lncRNA NEAT1 might regulate mRNA PGAP1, FKBP5 and CDON. The results of the quantitative real-time RT–PCR performed in ten other patients for NEAT1, PGAP1, FKBP5 and CDON were consistent with those of the sequencing analysis. Moreover, an in vitro experiment confirmed the interactions between NEAT1 and PGAP1, FKBP5 and CDON in human immortalized keratinocytes. Conclusion These findings suggest that the lncRNA–mRNA interaction network based on four pivotal nodes, NEAT1, PGAP1 FKBP5 and CDON, may play an important role in EMPD, which will contribute to a deeper understanding of the pathogenesis of EMPD.


2021 ◽  
pp. 1591-1595
Author(s):  
Takehisa Nakanishi ◽  
Makoto Kondo ◽  
Yasuo Nakai ◽  
Koji Habe ◽  
Keiichi Yamanaka

We present a case of extramammary Paget’s disease with bilateral inguinal lymph node metastasis treated by monthly docetaxel chemotherapy. He has also well-controlled psoriasis vulgaris for 20 years. One day after completing monthly chemotherapy, cellulitis by Group G <i>Streptococcus</i> occurred on both legs, resulting in septic shock and disseminated intravascular coagulation. During the infection, the tumor nodule volume and the exudate from the tumor decreased, and tumor markers carcinoembryonic antigen and cancer antigen 19-9 showed low values. Simultaneously, the psoriatic eruption reoccurred. We proposed that cytokine storm including tumor necrosis factor-alpha (TNF-α) during sepsis might have suppressed tumor lesions, and also TNF-α-dependent psoriatic rash appeared temporarily on his body.


2021 ◽  
Vol 11 ◽  
Author(s):  
Dan Zhao ◽  
Bo-ping Wen ◽  
Sen-yin Xu

Extramammary Paget’s disease (EMPD) is an uncommon intraepithelial malignancy that is rarely found in the male. Currently, there is very little knowledge pertaining to EMPD imaging, particularly in cases that involve the scrotum. Here, a 67-year-old man with lichenification on his left scrotum confirmed to be EMPD was reviewed. Bloodwork did not return a positive result, but syphilis-specific antibodies were found. Conventional high-frequency ultrasound (US) and contrast-enhanced ultrasound (CEUS) imaging were utilized to determine the lesion size and blood perfusion. In the present case, the lesion’s size and involvement were vividly depicted by CEUS, while results obtained by conventional US were grossly underestimated. Consequently, multimodal imaging assessment is likely to provide more accurate diagnoses for uncommon diseases, such as EMPD, and to aid in clinical decision-making.


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Liu Dong ◽  
Yingfeng Zhu ◽  
Liting Wu ◽  
Qiaoan Zhang ◽  
Feng Xu ◽  
...  

Background. Extramammary Paget’s disease (EMPD) is a rare skin tumor. Hypermethylation in the MSH2 promoter resulting in the downregulation of its protein expression shows a high detection rate in EMPD tumor tissue, which indicates that the methylation of MSH2 may play an important role in the pathogenesis of EMPD. Objective. This study aims to establish a rapid analysis strategy based on the methylation-sensitive high-resolution melting curve (MS-HRM) to detect the methylation level of the MSH2 promoter. Methods. With the use of universal methylated human DNA products, we established the MS-HRM standard curve to quantitatively detect the methylation level of the MSH2 promoter. Then, all 57 EMPD tumor DNA samples were analyzed. Pyrosequencing assay was also carried out to test the accuracy and efficacy of MS-HRM. Besides, a total of 54 human normal and other cancerous tissues were included in this study to test the reliability and versatility of the MS-HRM standard curve. Results. In this study, by using the established MS-HRM, we found that 96.5% (55/57) EMPD tumor samples had varying methylation levels in the MSH2 promoter ranging from 0% to 30%. Then, the methylation data were compared to the results obtained from pyrosequencing, which showed a high correlation between these two techniques by Pearson’s correlation (r = 0.9425) and Bland–Altman plots (mean difference = −0.1069) indicating that the methylation levels analyzed by MS-HRM were consistent with DNA pyrosequencing. Furthermore, in 23 normal and 31 other cancerous tissue samples, there were two colorectal cancer (CRC) tissues that tested MSH2 methylation positive (1% and 5%) which confirmed that our established MS-HRM can be widely applied to various types of samples. Conclusion. MS-HRM standard curve can be used for the detection of the methylation level of MSH2 in EMPD tumor samples and other cancerous tissues potentially, which presents a promising candidate as a quantitative assay to analyze MSH2 promoter methylation in routine pathological procedure.


PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0259408
Author(s):  
Fatima Noor ◽  
Muhammad Hamzah Saleem ◽  
Jen-Tsung Chen ◽  
Muhammad Rizwan Javed ◽  
Wafa Abdullah Al-Megrin ◽  
...  

2021 ◽  
Author(s):  
Soichiro Sawamura ◽  
Tselmeg Mijiddorj Myangat ◽  
Ikko Kajihara ◽  
Kenichiro Tanaka ◽  
Maho Ide ◽  
...  

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