Extramammary <scp>P</scp>aget's disease of the perianal region: a 20‐year experience

Rajendran, Saissan; Koh, Cherry; Solomon, Michael J.

doi:10.1111/ans.12814

Cited by 18 publications

(11 citation statements)

References 31 publications

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“…Perianal involvement comprises 20% of EMPD cases in both genders and has the highest incidence of related underlying malignancy . Reported rates of underlying malignancy range from 33% to 86% and are typically rectal or tubo‐ovarian cancers …”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Depending on the anatomic location involved, certain underlying malignancies may appear more frequently. For example, perianal lesions are more commonly associated with anal or colorectal carcinomas . Accordingly, depending on the gender of the patient, appropriate studies to be considered include mammography, pap smear, pelvic ultrasonography, cystoscopy, colonoscopy, and computed tomography of the abdomen and pelvis …”

Section: Screening For Malignancymentioning

confidence: 99%

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Extramammary Paget's disease: a review of the literature

2018

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Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy. The disease typically affects older individuals aged 60-80 years and is seen most frequently in postmenopausal Caucasian women and Asian men. EMPD exhibits a predilection for the genital and perianal regions and may be associated with an underlying carcinoma in adjacent organs. EMPD presents a challenge in both diagnosis and management. Often treated empirically as various dermatitides, the correct diagnosis is frequently delayed by many years. Following diagnosis, an extensive search for an associated malignancy should be initiated. If invasive disease is present on biopsy, a sentinel lymph node biopsy may guide further treatment. Mohs micrographic surgery appears to be superior to wide local excision when considering tissue sparing ability and disease recurrence. Nonsurgical interventions have also been investigated with varied results. Regardless of treatment method, long-term follow-up is recommended to monitor for local disease recurrence, development of internal malignancy, regional lymphadenopathy, or distant metastasis.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Section: Screening For Malignancymentioning

confidence: 99%

Extramammary Paget's disease: a review of the literature

2018

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“…This is because PPD tends to present a discontinuous spread of pagetoid cells in the epidermis beyond the clinically apparent margin, and the entire lesion is consequently very difficult to remove with no residual cells remaining [19]. As a consequence, according to the literature, after surgery, patients with noninvasive lesions must undergo periodic proctoscopies and digital examinations, while the follow-up of patients with invasive diseases should include CT scan for lung and liver evaluation as well as the examination of inguinal lymph nodes [20]. There are no randomized trials in the literature to demonstrate the effectiveness of adjuvant therapy for this type of disease [6].…”

Section: Discussionmentioning

confidence: 99%

Local Excision of a Mucinous Adenocarcinoma of the Anal Margin (Extramammary Paget’s Disease) and Reconstruction with a Bilateral V-Y Flap

Pagnanelli

Nardi

Martella

et al. 2019

Case Reports in Surgery

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The case of a 75-year-old female with invasive extramammary Paget's disease of the anal margin, without involvement of the anal canal, is reported. The patient underwent wide local excision of the lesion with reconstruction with a double V-Y flap, a biopsy of the inguinal sentinel node, and a laparoscopic temporary colostomy. No guidelines exist on the treatment of this rare disease, and both wide local excision and abdominoperineal resection have been proposed. In the present case, the absence of invasion of the anal canal, also confirmed by intraoperative biopsies on the resection margins, and of local lymph node metastasis, as confirmed by the sentinel lymph node biopsy, allowed a sphincter-sparing operation with good functional and oncological results.

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“…There is still no consensus regarding the optimal resection margin within the anal canal. Previous case reports suggested that resection of the anal canal mucosa up to 1 cm above the dentate line is important to ensure inclusion of the entire anal squamous epithelium 4 15 . However, the resection margin within the anal canal should be carefully determined as excessive resection may easily impair the postoperative anorectal function.…”

Section: Discussionmentioning

confidence: 99%

Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal

et al. 2016

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Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.

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Extramammary Paget's disease of the perianal region: a 20‐year experience

Abstract: PPD is a management challenge. Association with synchronous and metachronous carcinomas may not be as strong as initially thought. Surgery is the mainstay treatment with the need to balance between minimizing disease recurrence and functional sequelae from excessive tissue loss.

Cited by 18 publications

References 31 publications

Extramammary Paget's disease: a review of the literature

Extramammary Paget's disease: a review of the literature

Local Excision of a Mucinous Adenocarcinoma of the Anal Margin (Extramammary Paget’s Disease) and Reconstruction with a Bilateral V-Y Flap

Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal

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