Extramammary Paget’s Disease

O’Kelly, Fardod; Lundon, Dara; Hegarty, Paul K.

doi:10.1007/978-3-319-30046-7_21

Cited by 3 publications

(7 citation statements)

References 48 publications

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“…While the origin of EMPD is still in debate, EMPD largely affects those areas with a high apocrine gland concentration including the genital skin, axillae, and anus . The vulva is the most commonly affected region and accounts for nearly 65% of all cases of EMPD.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…The lesions of EMPD may be entirely asymptomatic or may produce variable degrees of pruritus, burning, or tenderness . Due to the nonspecific nature of symptoms, EMPD is generally not suspected early in its course and is empirically treated with corticosteroids or antifungals.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Biopsy analysis should include routine hematoxylin and eosin staining as well as immunohistochemical stains. The characteristic Paget cells are large, round, atypical intraepithelial cells containing pale‐staining vacuolated cytoplasm and prominent nuclei . The large cytoplasmic vacuoles are due to abundant mucin, which is easily recognized by histochemical stains such as PAS/alcian blue …”

Section: Histologic Evaluationmentioning

confidence: 99%

“…Due to its rarity, the exact incidence of EMPD is unknown . EMPD typically affects older individuals aged 60–80 years, with the highest incidence at 65 years of age .…”

Section: Introductionmentioning

confidence: 99%

“…EMPD typically affects older individuals aged 60–80 years, with the highest incidence at 65 years of age . The most commonly affected groups are postmenopausal Caucasian women and Asian men, with a female preponderance of 3:1 . Familial occurrence is quite rare, with only seven reported cases found within the Japanese and British literature combined …”

Section: Introductionmentioning

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: Clinical Manifestationsmentioning

confidence: 99%

Section: Histologic Evaluationmentioning

confidence: 99%

“…Due to its rarity, the exact incidence of EMPD is unknown . EMPD typically affects older individuals aged 60–80 years, with the highest incidence at 65 years of age .…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Extramammary Paget's disease: a review of the literature

2018

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Staged pelvic exenteration followed by oblique fleur-de-lis rectus abdominis myocutaneous (OFRAM) flap and keystone flap reconstruction for extramammary Paget’s disease

et al. 2021

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Extramammary Paget's disease (EMPD) is a rare, locally aggressive cutaneous adenocarcinoma which typically involves the anogenital region. The prognosis heavily depends on early diagnosis with definitive surgical treatment. To reduce local recurrence, wide radical resection with margin control and Moh's micrographic surgery are conventionally recommended. We describe a case of a 65-year-old female with extensive perineal EMPD involving the vulva, vagina, ectocervix, urethra, and anal canal, extending to the sacral region. She required pelvic exenteration and skin resection with 5-cm margins. A rectus abdominis myocutaneous flap (OFRAM) was designed with a fleur-de-lis skin paddle, with the vertical limb extended in an oblique fashion. We name this the OFRAM design and it allows maximal recruitment of abdominal tissue for the reconstruction of massive pelvic defects. The oblique limb obliterated the pelvic floor defect after the pelvic exenteration, while the horizontal limbs were used to resurface the perineal defect. One week later, after formal histology confirmed oncologic clearance, a gluteal keystone flap was used to resurface the remnant posterior defect. We discuss the disease pathology and reconstructive options in such cases. Level of Evidence: Level V, therapeutic study.

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The current consensus for the diagnostic and treatment of extramammary Paget’s disease

Emelyanova¹,

Zikiryakhodzhaev

Saribekyan³

et al. 2023

Vestnik dermatologii i venerologii

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Extramammary Paget's disease is a slow-growing cutaneous intraepithelial adenocarcinoma of the apocrine glands in the anogenital and axillary regions. The disease is extremely rare, affects predominantly postmenopausal women, and has nonspecific clinical caracteristics, so that it can take 2-10 years from the first clinical signs to diagnosis. The vulva and the perianal region are the most affected zones. Extramammary Pagets disease is clinically manifested as well-defined erythematous plaques with secondary changes, such as scaling, ulceration and even bleeding. Differential diagnosis is made between primary and secondary forms of Extramammary Pagets disease, candidiasis, contact dermatitis, Crohn's disease, eczema, erosive squamous lichen, purulent hydradenitis, Langerhans cell histiocytosis, sclerosing lichen, psoriasis, squamous cell carcinoma in situ, amelanocytic melanoma and mycosis fungoide. Treatment mainly includes the surgical component, as well as the use of photodynamic therapy, cytostatic agents, and, depending on the stage, systemic chemotherapy

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Extramammary Paget’s Disease

Cited by 3 publications

References 48 publications

Extramammary Paget's disease: a review of the literature

Extramammary Paget's disease: a review of the literature

Staged pelvic exenteration followed by oblique fleur-de-lis rectus abdominis myocutaneous (OFRAM) flap and keystone flap reconstruction for extramammary Paget’s disease

The current consensus for the diagnostic and treatment of extramammary Paget’s disease

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