Surgical Treatment for Extramammary Paget’s Disease

Wollina, Uwe; Goldman, Alberto; Bieneck, Andrzej; Abdel-Naser, Mohamed Badawy; Petersen, Sven

doi:10.1007/s11864-018-0545-x

Cited by 34 publications

(47 citation statements)

References 82 publications

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“…EMPD is an uncommon malignancy of the skin, classified as an intraepithelial adenocarcinoma that most frequently occurs in regions with an abundance of apocrine glands. The most commonly affected are the vulva, scrotum, and perianal areas [6]. EMPD represents 6.5% of all Paget's cases [7].…”

Section: Discussionmentioning

confidence: 99%

Recurrence of Noninvasive Perianal Extramammary Paget’s Disease in the Lymph Nodes without Local Recurrence after Complete Excision

Maeda

Minagawa

Koyama

et al. 2020

Case Rep Gastroenterol

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Primary extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma. Lymph node metastasis from noninvasive EMPD originating in the anorectal region is extremely rare, and the recurrence of noninvasive EMPD is commonly associated with local recurrence mainly due to an insufficient resection margin. We herein report a case of inguinal and para-aortic lymph node recurrence without local recurrence after complete margin-free surgical resection of noninvasive perianal EMPD. The patient was a man in his 40s who presented with an erythematous plaque of 7 × 5 cm in the perianal region, which had been present for 1 year. Biopsy from the perianal skin suggested EMPD; it was positive for cytokeratin (CK)7 and negative for CK20. Underlying malignancy was ruled out based on whole-body enhanced computed tomography (CT) and total colonoscopy. Surgery including complete wide resection of the lesion with preservation of the rectum was performed, and VY-advancement flap

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Section: Discussionmentioning

confidence: 99%

Recurrence of Noninvasive Perianal Extramammary Paget’s Disease in the Lymph Nodes without Local Recurrence after Complete Excision

Maeda

Minagawa

Koyama

et al. 2020

Case Rep Gastroenterol

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“…For example, topical drugs are indicated for noninvasive EMPD only. However, for invasive EMPD, surgical treatment is the preferred method in clinical practice . Regarding surgery, since EMPD often has irregular and undefined edges, it may be deeper or larger than the clinically determined extent.…”

Section: Discussionmentioning

confidence: 99%

Ultrasound Biomicroscopy and High‐Frequency Ultrasound for Evaluating Extramammary Paget Disease With Pathologic Correlation

Chen

Guo

Yan

et al. 2019

J of Ultrasound Medicine

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Objectives-The purpose of this study was to investigate the performance of ultrasound biomicroscopy (UBM) and high-frequency ultrasound (HFUS) in the assessment of extramammary Paget disease (EMPD) and to correlate the imaging features with pathologic findings.Methods-In this retrospective study, we described the imaging features from UBM and HFUS based on 17 pathologically proven EMPD cases. The performance for visualizing layer involvement by UBM and HFUS was compared. Additionally, we checked the consistency between layer involvement of the lesions on UBM images and the pathologic results. Additionally, blood flow and the status of lymph nodes were investigated with HFUS.Results-Ultrasound biomicroscopy revealed that all 17 lesions (100%) were hypoechoic and grew in a creeping form. The feature of layer involvement was shown in 10 lesions (58.8%) limited to the epidermis and 6 lesions (35.3%) involving the dermis, and the remaining lesion (5.9%) involved the full skin layers. Layer involvement was clearly displayed by UBM for all lesions (100%) but for only 5 lesions (29.4%) by HFUS (P < .001). Additionally, the layer involvement of 15 lesions (88.2%) on UBM was consistent with the pathologic results (κ = 0.746). High-frequency ultrasound revealed profuse blood flow in most lesions (64.7% [11 of 17]), and 1 case showed inguinal lymph node metastasis.Conclusions-Combined use of UBM and HFUS can provide key information on EMPD based on ultrasound features. Comparatively, UBM provides clearer morphologic information, whereas HFUS provides information on lymph node metastasis and blood flow.

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“…Paget's disease is a rare neoplasm of the nipple (1). Extramammary Paget's disease (EMPD), which was first described by Crocker in 1888 (1), is an intraepithelial adenocarcinoma (2) in apocrine cells rich glandular regions (1). It's more common in Cacausian women and Asian men (1).…”

Section: Introductionmentioning

confidence: 99%

“…Surgical treatment is an effective method in removal of premalignant lesions but in VIN, relapse is very common (2). EMPD and VIN alone are common lesions of vulve, but concurrence of these neoplasms in a same lesions, is a rare event (1,5).…”

Section: Introductionmentioning

confidence: 99%

Concurrence of Primary Cutaneous Extra Mammary Paget’s Disease and Squamous Cell Carcinoma in situ of Vulva: A Case Report

Samiee-Rad¹,

Farajee²

2020

Iran J Pathol

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Extramammary Paget's disease and vulvar intraepithelial neoplasia are common lesions of vulve, but synchronous occurrence is rare in a same location. Herein we describe a concurrence of primary cutaneous extra mammary Paget's disease and squamous cell carcinoma in situ of vulva in an Iranian women. A 59 year old woman, initially presented to Kosar teaching hospital gynecology clinic April 2017 with a single, well defined, scaly, white ulcerated mass, 2 cm in diameter on right minor labia, but other examinations were normal. She had no urinary or gastrointestinal symptoms. Incisional biopsies from the mass represented concurrence extramammary Paget's disease and VIN3. She underwent radical vulvectomy .No evidence of disease recurrence was noted after 16 months follow up. Participant consent was obtained before patient was enrolled in this study. There are lot of diseases that involve genitalia and lead to vulvar lesions. But in this rare case, we diagnosed concurrence of extramammary Paget's disease and vulvar intraepithelial neoplasia in a same location. Additionally, the clinical presentation as a vulvar mass was found unusual. Therefore, we report the case to sensitize gynecologists and pathologists for uncommon pathologies and their manifestations in vulva.

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Surgical Treatment for Extramammary Paget’s Disease

Cited by 34 publications

References 82 publications

Recurrence of Noninvasive Perianal Extramammary Paget’s Disease in the Lymph Nodes without Local Recurrence after Complete Excision

Recurrence of Noninvasive Perianal Extramammary Paget’s Disease in the Lymph Nodes without Local Recurrence after Complete Excision

Ultrasound Biomicroscopy and High‐Frequency Ultrasound for Evaluating Extramammary Paget Disease With Pathologic Correlation

Concurrence of Primary Cutaneous Extra Mammary Paget’s Disease and Squamous Cell Carcinoma in situ of Vulva: A Case Report

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