Evaluation of mapping biopsies for extramammary Paget disease: A retrospective study

doi:10.1016/j.jaad.2017.12.040

Journal of the American Academy of Dermatology

Volume 78, Issue 6, June 2018, Pages 1171-1177.e4

https://doi.org/10.1016/j.jaad.2017.12.040 Get rights and content

Background

Extramammary Paget disease (EMPD) sometimes shows an ill-defined border and an unexpectedly extended tumor spread beyond the clinical borders. Mapping biopsy is 1 approach for complete surgical removal, but its efficacy has remained controversial.

Objective

We sought to evaluate mapping biopsies for EMPD.

Methods

We performed a retrospective review of 133 patients with 150 primary EMPD lesions. We histopathologically examined 1182 skin biopsy specimens (975 from mapping biopsy and 207 from lesional biopsy).

Results

Only 1.6% of mapping biopsy specimens from well-defined EMPD (13 of 810) were positive. Moreover, 4.6% of mapping biopsy specimens from ill-defined EMPD (8 of 165) were positive, whereas all specimens taken from sites 2 cm or more from the clinical border were negative. For both well-defined and ill-defined EMPD, there was no significant difference in the margin status of surgical resection regardless of mapping biopsy.

Limitations

This was a retrospective study.

Conclusions

Mapping biopsies are unnecessary for well-defined EMPD or when 2-cm margins can be achieved, whereas surgical removal with predetermined margins (1 cm for well-defined EMPD and 2 cm for ill-defined EMPD) appears to be safe. Mapping biopsies can be considered when shortening of the safe surgical margin to less than 2 cm is required in ill-defined EMPD.

Section snippets

Patients

This study is a retrospective review of our patients that was conducted in accordance with the principles embodied in the Declaration of Helsinki and was approved by the Institutional Ethics Committee of Kyushu University (No. 26-1). We identified 133 patients with 150 EMPD lesions at the Department of Dermatology of Kyushu University (Fukuoka, Japan) between January 1997 and June 2016. All lesions were primary EMPD; cases of secondary EMPD were excluded. We retrieved clinical and demographic

Demographic and clinical data

Comprehensive clinical data on all 133 patients with 150 EMPD lesions are shown in Table I. All patients were Japanese, with a mean age of 72.0 years. Tumors were predominantly localized in the genital area (85.7%). A total of 15 patients (11.3%) had a double primary EMPD and 1 had a triple primary EMPD (Supplemental Table I; available at http://www.jaad.org). The median follow-up period was 6.9 years (range, 1.2 months–17.1 years). All of the patients underwent lesional biopsy to confirm the

Discussion

In the current study, we re-evaluated the efficacy of mapping biopsy and made several important findings. First, we detected Paget cells in only 21 (2.2%) of 975 mapping biopsy specimens. This rate of positivity for tumor seems to be extremely low and may indicate the limited importance of mapping biopsy in a clinical context. When we focused on mapping biopsy for well-defined EMPD, only 1.6% of biopsy specimens were affected by Paget cells, suggesting that mapping biopsy should be skipped for

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Cited by (31)

Extramammary Paget's disease: Our 15-year experience in a Southeast Asian population
2023, Journal of Plastic, Reconstructive and Aesthetic Surgery
The treatment of choice for Extramammary Paget’s disease (EMPD) is wide excision. However, owing to the tendency of microscopic spread and multi-centricity of the disease, resection margins are hard to determine. Despite the use of adjunctive methods such as mapping biopsy and Moh’s micrographic surgery, recurrence rates remain high. We aim to establish treatment guidelines by determining the variables associated with recurrence and the optimal resection margin size. We reviewed 52 patients who underwent wide excision in our institution between 2002 and 2017. A retrospective review of patient demographics, disease characteristics, and resection margins was performed. Most patients were Chinese (n = 39, 75%) male (n = 38, 73.1%). The mean tumor size was 6.73 cm (SD=4.10; range, 1.50–21.0 cm). The mean resection margin was 2.5 cm (SD=1.21; range, 0.20–5.50 cm). Eleven patients (21.2%) had disease recurrence. Nodal involvement significantly correlated with disease recurrence or mortality related to disease (HR=4.645; 95% CI=1.539,14.018; p = 0.0064). Subgroup analysis showed a significant correlation between resection margin size and recurrence rates (p = 0.047). We observed that a smaller resection margin (<2 cm) is acceptable for smaller tumor sizes (<6 cm) to achieve the lowest possible recurrence rates (20%), whereas a larger resection margin (>2 cm) is required for larger tumor sizes (>6 cm) (p = 0.012). Our results suggest that a resection margin recommendation can be made in correlation to the tumor size. This serves as a guideline for surgeons to predict the defect size and provide options for reconstructive surgery while achieving low recurrence rates.
Extramammary Paget's disease: Updates in the workup and management
2022, Asian Journal of Urology
Citation Excerpt :
Another case study by Adashek et al. [27] described using a punch biopsy-based mapping technique for determination of true borders of the lesion, by marking the skin surrounding the lesion and obtaining 2 mm punch biopsies circumferentially every 1 cm out to 5 cm from the visible edges of the lesion. Contrary to this approach, some have advocated for smaller margins between 1 cm and 2 cm [27,28]. Closer margins presented higher recurrence rates but could minimize wound defects that might require subsequent skin graft or perineal reconstruction procedure (Fig. 3) [29].
Extramammary Paget's disease (EMPD) is a rare cutaneous malignant disease. Due to its rarity, there is a paucity of data regarding best treatment strategy. EMPD primarily affects apocrine gland-bearing skin areas such as the vulva, scrotum, and penis. Our objective was to provide a present-day rationale for diagnosis, pathogenesis, and treatment of EMPD with a focus on recent progress in workup and management of the disease.
Literature on EMPD until February 2022 was assessed through PubMed, MEDLINE databases, and Google scholar. A narrative review of the most relevant articles was provided.
EMPD usually presents with indolent growth while usually being diagnosed primarily as carcinoma in situ. The foundation of EMPD treatment centers around prompt and accurate diagnosis, wide local or Mohs micrographic surgical excision with proper management towards the margin status, and careful consideration for lymphadenectomy in patients with regionally positive disease. Conventional chemotherapies are alternative treatments modality for patients with distant metastases; however, they sometimes have suboptimal efficacy. At present, there is no agreement regarding adjuvant or systemic therapies, although recent studies have shown several insights into the molecular pathogenesis, tumor biology, and genomics of the development and advancement of EMPD, which may lead to novel and targeted treatment approaches for metastatic EMPD in the future.
Patients with EMPD should seek care from physicians with expertise in disease management and patient counseling. These patients should be surveilled with close follow-up to evaluate them for disease recurrence or progression. Global collaborations with groups such as the Global Society for Rare Genitourinary Tumors, and especially patient support groups are crucial in designing clinical trials to help elucidate more robust data in this orphan disease.
Multidisciplinary Approach for the Management of Penoscrotal Extramammary Paget's disease –An eUROGEN study
2021, Urologic Oncology: Seminars and Original Investigations
Citation Excerpt :
However, the advanced age and comorbidities in this patient cohort often precludes offering further extensive surgery and adjuvant topical or laser treatment was used. The results of our case series which is the largest from a European centre are comparable to other published reports and based on these results we have proposed an algorithm for the management of patients with penoscrotal EMPD (Fig. 3) [1-40]. This multidisciplinary approach has meant that patients can be treated for non-invasive EMP and avoid extensive surgery provided that R1 resections are accepted and patients undergo adjuvant topical treatment and close surveillance.
We reviewed the medical and surgical management and long-term outcomes for patients diagnosed with penoscrotal extramammary Pagets disease (EMPD) within an eUROGEN centre.
Retrospective review of cases from an institutional database with biopsy proven penoscrotal EMPD.
A total of 10 patients were identified with penoscrotal EMPD over a 10-year period. Two patients had a previous history of gastrointestinal and urogenital cancers (20%) and no synchronous or metachronous cancers were identified. Eight patients with non-invasive EMPD (80%) underwent wide local excision of the affected skin, with at least a 5mm macroscopic resection margin and in selected cases simultaneous multiple mapping biopsies around the lesion were performed. Residual disease was present at the margins in seven patients (87.5%), of which three required further surgical excision or adjuvant topical immunotherapy (42.8%). Recurrence after complete excision was 12.5% and was successfully treated with topical imiquimod immunotherapy and CO₂ laser therapy. Two patients (20%) had invasive carcinoma and metastatic disease at diagnosis.
Reported recurrence rates of non-invasive penoscrotal EMPD are high and residual disease is present in most cases requiring either close clinical surveillance or adjuvant treatment. We propose an algorithm in the management of this rare disease.
Extramammary Paget Disease
2020, Actas Dermo-Sifiliograficas
Existen pocos estudios sobre la enfermedad de Paget extramamaria (EPEM) en la población mediterránea. Nuestro objetivo fue revisar las características de nuestros pacientes con EPEM, su asociación con neoplasia en continuidad y su evolución a largo plazo.
Realizamos un estudio observacional retrospectivo sobre 27 pacientes diagnosticados de EPEM entre 1990-2015. Las historias clínicas fueron revisadas retrospectivamente para obtener los datos clínico-patológicos y de seguimiento.
Se trata de 20 mujeres y 7 varones de entre 42 y 88 años de edad (mediana de 76 años). Las lesiones se localizaron en la vulva (16 casos), en el pubis-región inguinal (5), en la región perianal (4) y en la axila (2). El tiempo de evolución al diagnóstico osciló entre 1 y 60 meses (mediana de 12 meses) y el diámetro máximo entre 20 y 140 mm (mediana de 55 mm). En 3 casos (11,1%) la EPEM fue secundaria. Ningún caso se desarrolló sobre adenocarcinoma anexial cutáneo previo. Diez de 24 EPEM primarias (41,7%) presentaban invasión de la dermis. Ocho de los 27 pacientes (29,6%) presentaron recidiva local tras el tratamiento quirúrgico inicial. Tres pacientes (11,1%) fallecieron a consecuencia de metástasis de la EPEM.
La presencia de un adenocarcinoma anexial cutáneo subyacente es poco frecuente pero no es rara la existencia de un adenocarcinoma extracutáneo en continuidad. A pesar de que la EPEM suele evolucionar lentamente, es frecuente la invasión de la dermis y no son excepcionales las metástasis. Las recidivas locales son frecuentes a pesar de la extirpación con márgenes amplios y pueden ser tardías, por lo que es preciso un seguimiento a largo plazo.
Extramammary Paget disease (EMPD) has seldom been studied in Mediterranean populations. We aimed to review the characteristics of our patients with EMPD, the presence of a neoplasm in continuity, and the long-term course of the disease.
Retrospective observational study of 27 patients diagnosed with EMPD between 1990 and 2015. All clinical and pathology findings related to clinical course and outcomes were retrieved for analysis.
Twenty patients were women and 7 were men. Ages ranged from 42 to 88 years (median, 76 years). Lesions were in the following locations: vulva (16 cases), pubis–groin (5), perianal region (4), and axilla (2). Time from onset to diagnosis ranged from 1 to 60 months (median, 12 months) and maximum lesion diameter from 20 to 140 mm (median, 55 mm). In 3 cases (11.1%) EMPD was a secondary condition. None of the lesions developed on a previous cutaneous adnexal adenocarcinoma. Ten of the 24 primary EMPDs (41.7%) invaded the dermis. Eight of the 27 patients (29.6%) experienced local recurrence after the initial surgical treatment.Three patients (11.1%) died as a consequence of metastasis from the EMPD.
The presence of an underlying cutaneous adnexal adenocarcinoma is uncommon, but it is not unusual to find an extracutaneous adenocarcinoma in continuity. Although EMPD is a slow-growing tumor, dermal invasion is frequent and metastasis is not uncommon. Local recurrence is common even after excision with wide margins and may be delated, so long term follow-up is essential.
Extramammary Paget's Disease
2019, Hematology/Oncology Clinics of North America
Citation Excerpt :
Preoperative mapping biopsies were performed in 45 of these patients (68%), and use of mapping biopsy did not correlate with local recurrence (P = 1.000).16 There are many studies investigating the role (if any) of preoperative mapping biopsies, intraoperative frozen sections, and other techniques to maximize the efficacy of surgery, which may be particularly relevant when Mohs is not available.25–31 Processing the peripheral and deep margin via Mohs, while sending the central portion for conventional pathology in order to assess tumor depth for prognostic purposes, is also reasonable.26
Practical management of extramammary Paget's disease
2020, Piel

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: Drs Kaku-Ito and Ito contributed equally to this article.

: Funding sources: Supported in part by grants from the National Cancer Center, Japan; Ministry of Education, Culture, Sports, Science and Technology; and Ministry of Health, Labor and Welfare, Japan.

: Conflicts of interest: None disclosed.

View full text

Dermatologic surgeryEvaluation of mapping biopsies for extramammary Paget disease: A retrospective study

Background

Objective

Methods

Results

Limitations

Conclusions

Section snippets

Patients

Demographic and clinical data

Discussion

J Dermatol Sci

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Urol

Extramammary Paget's disease

BJOG

Mammary and extramammary Paget's disease

J Eur Acad Dermatol Venereol

Tumor thickness as a prognostic factor in extramammary Paget's disease

J Dermatol

Extramammary Paget's disease: treatment, prognostic factors and outcome in 76 patients

Br J Dermatol

Extramammary Paget's disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin

Eur J Dermatol

Dermatologic surgery
Evaluation of mapping biopsies for extramammary Paget disease: A retrospective study