Perianal

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How to Surveil Perianal Paget's Disease: A Case Report

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How to Surveil Perianal Paget's Disease: A Case Report

Perianal Paget’s disease (PPD) is a rare manifestation of extramammary Paget’s disease. It is characterized by the presence of malignant glandular epithelial cells within the squamous epithelium. There is a well-established but poorly understood association between PPD and underlying malignancy. Due to the rarity of the disease, there are no established guidelines for treatment or surveillance of PPD. We present the unusual case of a 73-year-old woman with primary PPD without an underlying malignant lesion. The rarity of the disease renders its management and surveillance an ongoing challenge. Our case of PPD without an underlying malignancy poses the question of the most appropriate surveillance for this rare disease.

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Network meta-analysis of treatments for perineal extramammary paget’s disease: Focusing on performance of recurrence prevention

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Network meta-analysis of treatments for perineal extramammary paget’s disease: Focusing on performance of recurrence prevention

Perineal extramammary paget’s disease (EMPD) is characterized with high recurrence rate. Although numerous therapeutic measures for this disease have been reported so far, it is unknown whether there is significant difference in their recurrence-preventing efficiency. This study aims to compare the recurrence outcomes of reported perineal EMPD treatments. We searched public databases of for published studies concerning perineal EMPD treatments. After screening by inclusion and exclusion criteria, we extracted the data relevant to recurrence rate, and conducted network meta-analysis (NMA) by using Bayesian random-effects approach. Our analysis included 29 previous studies (involving both male and female patients) and 11 treatment designs which are wide local excision (WLE), local excision (LE), Mohs micrographic surgery (MMS), radiotherapy (RT), radical vulvectomy (RV), photodynamic therapy (PDT), lasers (LS), imiquimod, and WLE+RT, WLE+PDT, WLE+LS. MMS has the best performance on reducing perineal EMPD recurrence.

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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.

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.

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.

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Anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report

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Anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report

Anal canal cancer occasionally accompanies extramammary Paget disease. Although most of them are squamous cell carcinoma, anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease has never been reported.

This is a clinically significant case, as it reveals novel pathological features about anal canal cancer with secondary Paget disease and successfully treated with modified FOLFOX6. Careful pathological investigation and appropriate treatment choice are needed for this rare cancer.

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Wide local excision of perianal Paget’s disease with gluteal flap reconstruction: an interdisciplinary approach

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Wide local excision of perianal Paget’s disease with gluteal flap reconstruction: an interdisciplinary approach

Perianal Paget’s disease (PPD) is an extremely rare condition characterized as intraepithelial adenocarcinoma of unclear etiology. It can be either primary or secondary. The disease typically presents as an eczema-like, erythematous, and painful skin lesion that is associated with pruritus. It is usually misdiagnosed as a common anorectal problem. Surgical excision is the preferred treatment of PPD, with the specific technique being dependent upon disease invasiveness. 

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Perianal extramammary paget's disease associated with primary linitis plastica of the rectum: Report of a case

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Perianal extramammary paget's disease associated with primary linitis plastica of the rectum: Report of a case

We report herein a case of perianal extramammary Paget's disease associated with primary linitis plastica of the rectum. An 82-year-old woman was admitted to our hospital for investigation and treatment of a perianal eczematous lesion. A skin biopsy of the lesion revealed perianal extramammary Paget's disease and a barium enema demonstrated diffuse narrowing with an irregular contour at the ampulla recti. Under a suspected diagnosis of linitis plastica of the rectum, an abdominoperineal resection was performed to resect both the rectal and perianal lesions with regional lymphadenectomy. Grossly, marked narrowing and wall thickness were observed at the lower rectum, and a histological diagnosis of signet ring cell carcinoma was confirmed. The perianal eczematous lesion revealed many atypical cells with clear cytoplasm, being Paget cells, throughout the entire epidermis. Sparse distributions of signet ring cells were also observed in the subcutaneous tissue beneath the perianal eczematous lesion. The pathogenesis of perianal extramammary Paget's disease in this patient was therefore considered to be an intraepidermal extension of primary linitis plastica of the rectum.

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