Three-dimensional evaluation of subclinical extension of extramammary Paget disease: visualization of the histological border and its comparison to the clinical border

Murata, Takaho; Honda, T.; Egawa, Gyohei; Kitoh, Akihiko; Dainichi, Teruki; Otsuka, Atsushi; Nakajima, Saeko; Kore-Eda, Satoshi; Kaku, Yo; Nakamizo, Satoshi; Endo, Yuichiro; Fujisawa, Akihiro; Kabashima, Kenji

doi:10.1111/bjd.15282

Cited by 28 publications

(18 citation statements)

References 39 publications

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“…EMPD affects apocrine gland-rich sites such as the anogenital area and axillae. Despite requiring broad local resection for the treatment of a primary lesion because of the frequent microscopic extensions and less common satellite lesions beyond the clinical tumor border ( 1 ), the prognosis of patients with EMPD is usually good because tumor cells are in the radial growth phase for a prolonged duration, and a majority of cases are treated in the stage of carcinoma in situ ( 2 ).…”

Section: Invasive Extramammary Paget’s Disease (Empd) Therapeutic Chamentioning

confidence: 99%

Metastatic Extramammary Paget’s Disease: Pathogenesis and Novel Therapeutic Approach

Fukuda

Funakoshi

2018

Front. Oncol.

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Extramammary Paget’s disease (EMPD) is a rare, slow-growing, cutaneous adenocarcinoma that usually originates in the anogenital area and axillae outside the mammary glands. EMPD mostly progresses slowly and is often diagnosed as carcinoma in situ; however, upon becoming invasive, it promptly and frequently metastasizes to regional lymph nodes, leading to subsequent distant metastasis. To date, several chemotherapy regimens have been used to treat metastatic EMPD; however, they present limited effect and patients with distant metastasis exhibit a poor prognosis. Recently, basic and translational investigative research has elucidated factors and molecular mechanisms underlying the promotion of metastasis, which can lead to targeted therapy-based emerging treatment strategies. Here, we aim to discuss current therapies and their limitations; advancements in illustrating mechanisms promoting invasion, migration, and proliferation of EMPD tumor cells; and future therapeutic approaches for metastatic EMPD that may enhance clinical outcomes.

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Section: Invasive Extramammary Paget’s Disease (Empd) Therapeutic Chamentioning

confidence: 99%

Metastatic Extramammary Paget’s Disease: Pathogenesis and Novel Therapeutic Approach

Fukuda

Funakoshi

2018

Front. Oncol.

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“…The increase in the number of stages of MMS after topical application indicates the likelihood of a false-negative result by interfering with tumor continuity in the case of multifocal lesions, thereby leading to recurrence. However, there is a rare incidence of subclinical extension of small satellite lesions, 16 and sometimes these lesions can be hard to distinguish from recurreneces in the retrospective TA B L E 4 Clinical characteristics of three patients with compatible response to topical monotherapy negatively affected the tumor continuity, thereby resulting in a higher recurrence rate.…”

Section: Discussionmentioning

confidence: 99%

Initial topical monotherapy may increase the risk of recurrence in patients with extramammary Paget’s disease

Choi

Roh

et al. 2021

The Journal of Dermatology

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“…In EMPD, there are at least two patterns of subclinical extension: continuous and satellite lesions [10] . Subclinically extended Paget cells in small foci are likely to be invisible and are detected outside hypopigmented patches with erythema, whereas small satellite lesions are found only in the vicinity of the main lesions 11 .…”

Section: Discussionmentioning

confidence: 99%

Axillary Paget Disease with a Visible Satellite: A Case Report and Literature Review

Luo

Wang

et al. 2021

Preprint

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Background : Extramammary Paget disease is an uncommon malignancy affecting apocrine gland–bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside the clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. The standard management strategy for EMPD without distant metastasis is a complete surgical removal, sometimes called wide local excision. However, there is no consensus regarding surgical margin width to decrease the high recurrence rate. Here we describe the first macroscopically visible satellite of axillary EMPD and wide local excision of both main lesion and the satellite lesion with only 0.5cm margin, succeeded by a short review of the literature. Case presentation: A 48-year-old female with a red macule in the right axilla was presented to our clinic. A well-demarcated 4 cm×3 cm erythematous plaque was observed in the right axilla, and a similar lesion measuring 0.5 cm×0.3 cm was found 3.5 cm away from the primary site. Breast and axillary node examination was unremarkable. Biopsy of the large plaque revealed Paget disease, then we performed a local extended excision of both lesions with a 0.5cm margin, all margins negative indicated, by frozen pathology. Pathology revealed the nature of the satellite beside the main lesion also as Paget disease. The patient is currently followed-up for 20 months and has shown no signs of recurrence, with normal shoulder motor function. Conclusion: We have report the first visible satellite of extramammary Paget disease, indicating the necessity of an extended local resection of both the main leision and the satellite lesion. Considering the anatomical structure of axillary Paget disease, a 0.5cm negative surgical margin indicated by frozen pathology might be sufficient to sustain the shoulder motor function.

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Three-dimensional evaluation of subclinical extension of extramammary Paget disease: visualization of the histological border and its comparison to the clinical border

Abstract: In EMPD, at least two patterns of subclinical extension exist: continuous and satellite lesions. Subclinical extension might exist preferentially outside hypopigmented patches with erythema.

Cited by 28 publications

References 39 publications

Metastatic Extramammary Paget’s Disease: Pathogenesis and Novel Therapeutic Approach

Metastatic Extramammary Paget’s Disease: Pathogenesis and Novel Therapeutic Approach

Initial topical monotherapy may increase the risk of recurrence in patients with extramammary Paget’s disease

Axillary Paget Disease with a Visible Satellite: A Case Report and Literature Review

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