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

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yu-Wei Chang ◽  
Hsu Ma ◽  
Wen-Chieh Liao

Abstract Background 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. Methods We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Independent variables included patients’ demographic data, concurrent malignancy (i.e., non-EMPD-related cancers), tumor size, distant metastasis, and surgery and/or radiation. Results Of all the 63 patients, 8 cases were excluded. A total of 43 patients (78.18%) were male, and 12 were female, with a mean age of 72.67 years (range 44–89 years). The most common affected anatomic site was the penoscrotal region (22 patients, 40.00%), followed by the perianal and perineal regions (17 patients, 30.91%). Among the 55 patients, 41 patients (74.55%) were diagnosed with at least one underlying disease, whereas the most common underlying disease was cardiovascular disease (30 patients, 54.55%). The overall survival rate was 80.00% at 36 months and 65.45% at the end of follow-up. EMPD with deep dermal invasion was a significant poor prognostic factor of overall survival in cause-specific hazard model (sub-hazard ratio (HR) 5.167, p = 0.0015, 95% confidence interval (CI) 1.876–14.230). Patients with regional metastasis or distant metastasis had poorer prognosis of 5-year survival (sub-HR 4.513, p = 0.0028, CI 1.683–12.103). The limitations of this study include its retrospective nature and sample size. Conclusions 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.

2020 ◽  
Author(s):  
Yu-Wei Chang ◽  
Hsu Ma ◽  
WEN-CHIEH LIAO

Abstract Background: 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.Methods: We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Independent variables included patients’ demographic data, concurrent malignancy (i.e., non-EMPD-related cancers), tumor size, distant metastasis, and surgery and/or radiation.Results: Of all the 63 patients, 8 cases were excluded. A total of 43 patients (78.18%) were male, and 12 were female, with a mean age of 72.67 years (range: 44–89 years).The most common affected anatomic site was the penoscrotal region (22 patients, 40.00%), followed by the perianal and perineal regions (17 patients, 30.91%). Among the 55 patients, 41 patients (74.55%) were diagnosed with at least one underlying disease, whereas the most common underlying disease was cardiovascular disease (30 patients, 54.55%). EMPD with deep dermal invasion was a significant poor prognostic factor of overall survival in multivariate Cox regression (hazard ratio(HR): 5.167, p = 0.0015). The overall survival rate was 80.00% at 36 months and 65.45% at the end of follow up. The patients 75 years old or older had poorer outcome than those younger than 65 years old (HR: 7.676, p = 0.0496). Patients with regional metastasis or distant metastasis had poorer prognosis of 5-year survival (HR: 4.020 and 5.147, p = 0.0320 and 0.0118, respectively). The limitations of this study include its retrospective nature and sample size.Conclusions: In our series, EMPD with metastasis and deep dermal invasion were 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.


Diagnostics ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1086
Author(s):  
Shun Ohmori ◽  
Yu Sawada ◽  
Natsuko Saito-Sasaki ◽  
Sayaka Sato ◽  
Yoko Minokawa ◽  
...  

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.


Neurosurgery ◽  
2011 ◽  
Vol 70 (5) ◽  
pp. 1081-1094 ◽  
Author(s):  
Miran Skrap ◽  
Massimo Mondani ◽  
Barbara Tomasino ◽  
Luca Weis ◽  
Riccardo Budai ◽  
...  

Abstract BACKGROUND: Despite intraoperative technical improvements, the insula remains a challenging area for surgery because of its critical relationships with vascular and neurophysiological functional structures. OBJECTIVE: To retrospectively investigate the morbidity profile in insular nonenhancing gliomas, with special emphasis on volumetric analysis of tumoral resection. METHODS: From 2000 to 2010, 66 patients underwent surgery. All surgical procedures were conducted under cortical-subcortical stimulation and neurophysiological monitoring. Volumetric scan analysis was applied on T2-weighted magnetic resonance images (MRIs) to establish preoperative and postoperative tumoral volume. RESULTS: The median preoperative tumor volume was 108 cm3. The median extent of resection was 80%. The median follow-up was 4.3 years. An immediate postoperative worsening was detected in 33.4% of cases; a definitive worsening resulted in 6% of cases. Patients with extent of resection of > 90% had an estimated 5-year overall survival rate of 92%, whereas those with extent of resection between 70% and 90% had a 5-year overall survival rate of 82% (P < .001). The difference between preoperative tumoral volumes on T2-weighted MRI and on postcontrast T1-weighted MRI ([T2 − T1] MRI volume) was computed to evaluate the role of the diffusive tumoral growing pattern on overall survival. Patients with preoperative volumetric difference < 30 cm3 demonstrated a 5-year overall survival rate of 92%, whereas those with a difference of > 30 cm3 had a 5-year overall survival rate of 57% (P = .02). CONCLUSION: With intraoperative cortico-subcortical mapping and neurophysiological monitoring, a major resection is possible with an acceptable risk and a significant result in the follow-up.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 19508-19508
Author(s):  
S. A. Saravanan ◽  
V. Sokolovski ◽  
V. Voloshin ◽  
M. Aliev ◽  
V. Zybikov ◽  
...  

19508 Background: To analyse the five-year survival rate in patients with proximal femoral tumours after total hip replacement. Methods: Between the period of 1994–2003, 50 patients were operated (Total Hip Replacement) for proximal femoral tumours at the Department of General Oncology (Bone & Soft tissue tuomurs), N. N. Blokhin Cancer Research Institute & Moscow Regional Clinical Research Institute, Moscow, Russian Federation. The histological diagnoses included 14 - metastases, 10 - osteosarcoma, 8 - chondrosarcoma, 4 - Ewing’s sarcoma, 4 - Giant cell tumor, 3 - malignant fibrous histiocytoma, 2 paraosteal and 2 periosteal osteosarcoma, and 1 each from primary neuroectodermal tumor, myeloid disease, and aneurysmal bone cyst. The follow-up ranged from 1–9 years (mean follow-up 5 years). 21 patients (45.7%) had pathological fracture. The cause of the pathological fracture was metastasis in 12 patients (26%). 28 patients (60.8%), had soft tissue invasion. All the survival analyses were done using Kaplan-Meier survivorship analysis method. Functional outcome was estimated using Enneking’s evaluation criteria. Results: The overall survival rate of patients was 66.7% at 5 years. 2 patients had local recurrence.12 patients had metastases after surgery. In that 11 patients were died. There was no evidence of disease in 32 patients. In 3 patients, we performed disarticulation of the hip joint because of the local recurrence. The overall survival rate of limb was 92.7% at 5 years. The overall survival rate of prostheses was 84.2% at 5 years. At the latest follow up, functional outcome was excellent in 15 (30%) patients, good in 27 (54%) patients, fair in 5 (10%) patients, poor in 3 (6%) patients. Conclusions: Though the extent of the muscle and bone resection is large, there is no doubt that endoprosthetic replacement of the proximal femur provides a good functional and oncological outcome when compared with the various other reconstructive surgeries. No significant financial relationships to disclose.


2015 ◽  
Vol 33 (3_suppl) ◽  
pp. 144-144
Author(s):  
Boo Gyeong Kim ◽  
Byung-Wook Kim ◽  
Joon Sung Kim ◽  
Sung Min Park ◽  
Keun Joon Lim ◽  
...  

144 Background: The aim of this study is to evaluate the long-term clinical and oncologic outcome of ESD for differentiated EGC of an expanded indication compared to surgical resection. Methods: Retrospective analyses were performed in patients who underwent ESD or surgical resection for EGC of an expanded indication from 2006 and 2008 in Incheon St. Mary’s Hospital, Seoul St. Mary’s Hospital, Yeouido St. Mary’s Hospital, and St. Paul’s Hospital. First arm study was performed according to pre-ESD diagnosis including pathologic diagnosis and endoscopic findings. Second arm study was obtained from post-ESD final pathologic result. All the patients were checked with endoscopy and stomach CT regularly at least 5 years. Clinical outcomes, disease free survival and overall survival were compared between the ESD group and surgical resection group in each arm. Results: In first arm study, 41 patients who received ESD and 106 patients who received surgical resection were enrolled. Metachronous recur was found in 4 patients among ESD group and in 2 patients among surgical resection group during the follow up period. There was no local recurrence in both groups. The disease free survival was not different between the two groups (ESD vs surgical resection; 87.8 vs 95.3%, p=0.291). The 5-year overall survival rate was 100% in both groups. In second arm study, 74 patients who received ESD and 165 patients who received surgical resection were enrolled. Metachronous recur was found in 5 patients among ESD group and in 2 patients among surgical resection group during the follow up period. Local recurrence did not occur in both groups. Surgical resection group was superior to ESD group in disease free survival (97.6% vs 87.6%, p=0.002). The 5-year overall survival rate was 100% in both groups. Conclusions: ESD for EGC might be acceptable considering the overall survival rates. However, intensive surveillance should be performed to find the metachronous recur after ESD.


PLoS ONE ◽  
2021 ◽  
Vol 16 (7) ◽  
pp. e0254678
Author(s):  
Fatima Noor ◽  
Muhammad Hamzah Saleem ◽  
Jen-Tsung Chen ◽  
Muhammad Rizwan Javed ◽  
Wafa Abdullah Al-Megrin ◽  
...  

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.


1996 ◽  
Vol 114 (6) ◽  
pp. 1298-1302 ◽  
Author(s):  
Flávio Xavier ◽  
Lucélia de Azevedo Henn ◽  
Oliveira Marja ◽  
Luciane Orlandine

The frequency of smoking among patients with primary lung cancer diagnoses admitted to the Hospital de Clinicas de Porto Alegre (HCPA) during the 1980's was investigated. The objective of this study was to analyze cigarette consumption patterns through the number of cigarettes smoked per day and the age at which smoking began, correlating this data to the overall survival rate and histological type of the lung cancer. Methods: This retrospective study analyzed patients with primary lung cancer diagnosed at the HCPA between January 1980 and December 1989. All patients considered underwent follow-up for at least three years. Patient information was obtained either from the hospital's records or by contacting patients via letter or phone. Results: More than 90 percent of the patients were smokers or had smoked previously; most had started smoking before the age of 20.The overall 24-month survival rate after diagnosis varied depending on whether the patient had smoked less than 40 cigarettes per day or not. The percentage of smokers and non-smokers was established for each histological type, with the bronchoalveolar adenocarcinoma type showing the highest percentage of non-smokers (40 percent). Conclusion:The overall survival rates of patients with lung cancer was related to the number of cigarettes smoked, and not to the fact of the patient having smoked or not.The number of smokers among patients with lung cancer was not so high only for the bronchoalveolar adenocarcinoma histological type.


2021 ◽  
Author(s):  
Xiang Gao ◽  
Xi Zhong ◽  
Hai-Ning Chen ◽  
Dujanand Singh ◽  
Lie Yang ◽  
...  

Aim: To develop nomograms for predicting cancer-specific survival (CSS) and overall survival (OS) in patients with invasive extramammary Paget’s disease (iEMPD). Patients & methods: Retrospective data of 1955 patients with iEMPD were collected from the Surveillance, Epidemiology, and End Results database. Nomograms for predicting CSS and OS were established using competing risk regression and Cox regression, respectively, and were internally validated. Results: Five (age, surgery, tumor location, stage and concurrent malignancy) and eight (gender, age, race, marital status, surgery, tumor location, stage and lymph node metastasis) clinicopathological factors were utilized to construct nomograms for predicting CSS and OS, respectively. The concordance indices of the nomograms for predicting CSS and OS were 0.78 and 0.73, respectively. The validation of the nomograms showed good calibration and discrimination. The decision curve analyses confirmed the clinical utility of these nomograms. Conclusion: The nomograms can be a reliable tool for treatment design and prognostic evaluation of iEMPD.


1996 ◽  
Vol 63 (1) ◽  
pp. 50-54
Author(s):  
F. Pagano ◽  
A. Tasca ◽  
C.A. Levorato

— Of the 176 patients treated in our Institute over the last 16 years for upper tract transitional cell carcinoma (UTTCC), 155 were included in this study, 97 of whom had undergone nephroureterectomy, 28 conservative surgery and 30 endourologic treatment. The overall survival rate at 5 and 10 years was 73% and 58%, respectively. Univariate analysis of overall survival rate (O.S.) and disease-free survival rate (D.F.S.) showed no difference between patients with superficial and with invasive tumours, while multivariate analysis highlighted a difference. No difference was evident upon univariate and multivariate analysis of the patients with superficial tumours who underwent conservative (19) vs. radical (45) surgery. The same can be said for the so-called good prognosis tumours (pTa-T1-G2) and poor prognosis tumours (pT1-G3, pT2-4, G2-3) submitted to radical surgery. Overall survival was better in patients with no recurrent tumours compared to those with recurrences; furthermore prognosis was worse for patients with more than 2 recurrences. Tumour site, mono or multifocality and associated bladder tumour did not influence prognosis. Our experience confirms the basic importance of grade as a prognostic factor for UTTCC and the favourable prognosis for patients with superficial tumours, regardless of surgical technique. A rigid follow-up is mandatory for patients with more than 2 recurrences.


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