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

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.

Prognostic Factors of Extramammary Paget’s Disease: A Retrospective Study at a Medical Center in Taiwan

“…The 5-year recurrence rate (33%) in our study was much higher compared with other EMPD studies that treated patients initially with wide local excision (2,12,13). Intra-operation frozen sections being performed only in 5 patients (26.32%) is considered to be the main cause.…”

A Systematic Review and Meta-Analysis of Mapping Biopsy for Primary Extramammary Paget’s Disease in Reducing Recurrence Following Surgical Excision

“…Updated literature has yet to find a correlation between free-margin status and recurrence-free survival following surgical excision. 26 , 27 …”

Value of pseudopod sign on high‐frequency ultrasound in predicting the pathological invasion of extramammary Paget's disease lesions

“…Similar to the findings of previous studies, EMPD has a predilection for older Asian males and occurs usually around their genitalia. [22][23][24] The early stage of EMPD is in situ and often confused with chronic inflammatory dermatosis, such as eczema, 12 frequently leading to a delay in diagnosis and treatment. The lesion may gradually invade deeper directly or through the adnexal area, and even cause distant metastasis.…”

“…Second, the lesion was evaluated in detail (thickness, layer involvement, pseudopodia, blood perfusion, etc.) with a high-frequency transducer (22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38) MHz; KOLO, Jiangsu, China). For some extremely thin lesions, we used an ultrasound biomicroscope (UBM) equipped with a mechanical scanning line array transducer (50 MHz, MD-300SII; Meda Co., Ltd., Tianjin, China) to explore the aforementioned details.…”