Extramammary Paget's disease: Surgical treatment with Mohs micrographic surgery

doi:10.1016/j.jaad.2004.07.004

Journal of the American Academy of Dermatology

Volume 51, Issue 5, November 2004, Pages 767-773

https://doi.org/10.1016/j.jaad.2004.07.004 Get rights and content

Background

Extramammary Paget's disease (EMPD) is an uncommon tumor that has a high rate of recurrence after conventional surgical treatments.

Objective

Our purpose was to establish the efficacy of Mohs micrographic surgery (MMS) in the treatment of EMPD, and to make treatment recommendations with regard to surgical margins. We also attempted to summarize the published recurrence rates of EMPD after standard surgical management.

Methods

In a retrospective chart review, pertinent demographic data, tumor data, treatment characteristics, and follow-up data were tabulated. A search of the literature for recurrence rates after MMS and non-MMS surgical treatment modalities was performed.

Results

The recurrence rate after treatment with MMS was 16% for primary EMPD and 50% for recurrent EMPD. The 5-year tumor-free rates (Kaplan-Meier analysis) were 80% for primary tumors and 56% for recurrent tumors. Using MMS, the salvage rate (and, hence, overall cure rate) was 100%. Margins of 5 cm were required to clear 97% of the tumors. The recurrence rate after non-MMS (from the literature) is 33% to 60%.

Conclusion

MMS is effective, and superior to standard surgical management in the treatment of EMPD. We recommend a 5-cm margin of normal skin if MMS cannot be offered.

Section snippets

Methods

Daily surgical logs for all the cases performed by the authors (J. A. Z.: 1983-2003; D. G. B.: 1997-2003) were reviewed and all cases of EMPD were noted. Detailed review of each patient's medical record was undertaken and all relevant data collected (Table I). Follow-up visits and telephone surveys were performed for all patients. Examination of the affected area by a physician was required to establish the presence or absence of recurrence. Recurrence was defined as the

Results

In all, 25 patients with 27 lesions of EMPD were referred for MMS. There were 12 male and 13 female patients. All patients were Caucasian. Of the tumors, 52% (14/27) involved the male gentialia/groin (including one perianal). The remaining 13 tumors were in women and consisted of 26% (7/27) vulvar, 11% (3/27) axillary, 7% (2/27) perianal, and 4% (1/27) pubic.

A total of 34 cases of MMS were performed on 19 primary tumors, 8 tumors recurrent after non-MMS, and 7 tumors recurrent after MMS. Table I

Discussion

It is clinically difficult to distinguish the margins of EMPD from normal skin. Standard surgical management of EMPD are associated with an inherently high recurrence rate, and repeated operations lead to significant morbidity and deformity. MMS offers an alternative to blind excision of EMPD, or excision with frozen-section sampling of a fraction of the margins. It offers the ability to microscopically visualize the entire margin and remove only the affected tissue and, hence, a lower

References (22)

R.F. Wagner et al.
Treatment of extensive extramammary Paget disease of male genitalia with Mohs micrographic surgery
Urology
(1988)
J. Fanning et al.
Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision
Am J Obstet Gynecol
(1999)
J.J. Chanda
Extramammary Paget's disease: prognosis and relationship to internal malignancy
J Am Acad Dermatol
(1985)
R.E. Jones et al.
Extramammary Paget's disease: a critical reexamination
Am J Dermatopathol
(1979)
C.M. Whorton et al.
Carcinoma of moll's glands with extramammary Paget's disease of the eyelid
Cancer
(1995)
C.V. Wetli et al.
Tumors of ceruminous glands
Cancer
(1972)
B. Remond et al.
Umbilical Paget's disease and prostatic carcinoma
Br J Dermatol
(1993)
P. Marchesa et al.
Long-term outcome of patients with perianal Paget's disease
Ann Surg Oncol
(1997)
J.D. Zollo et al.
The Roswell Park Cancer Institute experience with extramammary Paget's disease
Br J Dermatol
(2000)
F.E. Mohs et al.
Microscopically controlled surgery for extramammary Paget's disease
Arch Dermatol
(1979)

B.M. Coldiron et al.

Surgical treatment of extramammary Paget's disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision

Cancer

(1991)

Cited by (229)

Review on the Role of Paraffin-embedded Margin-controlled Mohs Micrographic Surgery to Treat Skin Tumors
2024, Actas Dermo-Sifiliograficas
Paraffin-embedded margin-controlled Mohs micrographic surgery (PMMS) includes various procedures such as slow Mohs or deferred Mohs technique, the Muffin and Tübingen techniques, and staged margin excision, or the spaghetti technique. PMMS is a variation of conventional Mohs micrographic surgery (MMS) that allows histopathological examination with delayed margin control. PMMS requires minimum training and may be adopted by any hospital. The setback is that PMMS can require procedures across multiple days. PMMS lowers the rate of recurrence of basal cell carcinoma vs wide local excision in high-risk basal cell carcinoma, and improves the rates of recurrence and survival in lentigo maligna. PMMS can be very useful in high-risk squamous cell carcinoma treatment. Finally, it is a promising technique to treat infrequent skin neoplasms, such as dermatofibrosarcoma protuberans, or extramammary Paget's disease, among others. In this article, we present a literature narrative review on PMMS, describing techniques and indications, and highlighting long-term outcomes.
La cirugía micrográfica con márgenes controlados en parafina (PMMS) incluye diversos procedimientos como el slow Mohs o Mohs en diferido, la técnica del Muffin, la torta de Tübingen, y la escisión de márgenes por etapas o espagueti. La PMMS es una modificación de la cirugía de Mohs convencional (MMS) con estudio histológico en diferido. La PMMS requiere una formación mínima y podría ejecutarse en cualquier hospital. Como inconveniente, la PMMS puede requerir realizar procedimientos en distintos días. La PMMS ha demostrado una tasa de recurrencia más baja en comparación con la exéresis convencional en el carcinoma basocelular, y una menor tasa de recurrencia y supervivencia más prolongada en el lentigo maligno. La PMMS también es de gran utilidad en el carcinoma de células escamosas, y en tumores infrecuentes. En este artículo, presentamos una revisión narrativa sobre la PMMS, describiendo técnicas e indicaciones, y destacando sus resultados a largo plazo.
Mohs surgery for female genital Paget's disease: a prospective observational trial
2023, American Journal of Obstetrics and Gynecology
Extramammary Paget’s disease recurs often after traditional surgical excision. Margin-controlled surgery improves the recurrence rate for male genital disease but is less studied for female anatomy.
This study aimed to compare surgical and oncologic outcomes of margin-controlled surgery vs traditional surgical excision for female genital Paget’s disease.
We conducted a prospective observational trial of patients with vulvar or perianal Paget’s disease treated with surgical excision guided by Mohs micrographic surgery between 2018 and 2022. The multidisciplinary protocol consisted of office-based scouting biopsies and modified Mohs surgery followed by surgical excision with wound closure under general anesthesia. Modified Mohs surgery cleared peripheral disease margins using a moat technique with cytokeratin 7 staining. Medial disease margins (the clitoris, urethra, vagina, and anus) were assessed using a hybrid of Mohs surgery and intraoperative frozen sections. Surgical and oncologic outcomes were compared with the outcomes of a retrospective cohort of patients who underwent traditional surgical excision. The primary outcome was 3-year recurrence-free survival.
Three-year recurrence-free survival was 93.3% for Mohs-guided excision (n=24; 95% confidence interval, 81.5%–100.0%) compared to 65.9% for traditional excision (n=63; 95% confidence interval, 54.2%–80.0%) (P=.04). The maximum diameter of the excisional specimen was similar between groups (median, 11.3 vs 9.5 cm; P=.17), but complex reconstructive procedures were more common with the Mohs-guided approach (66.7% vs 30.2%; P<.01). Peripheral margin clearance was universally achieved with modified Mohs surgery, but positive medial margins were noted in 9 patients. Reasons included intentional organ sparing and poor performance of intraoperative hematoxylin and eosin frozen sections without cytokeratin 7. Grade 3 or higher postoperative complications were rare (0.0% for Mohs-guided excision vs 2.4% for traditional excision; P=.99).
Margin control with modified Mohs surgery significantly improved short-term recurrence-free survival after surgical excision for female genital Paget’s disease. Use on medial anatomic structures (the clitoris, urethra, vagina, and anus) is challenging, and further optimization is needed for margin control in these areas. Mohs-guided surgical excision requires specialized, collaborative care and may be best accomplished at designated referral centers.
Topical Calcipotriol Plus Imiquimod Immunotherapy for Nonkeratinocyte Skin Cancers
2023, JID Innovations
Nonkeratinocyte cutaneous malignancies, including breast cancer cutaneous metastasis and melanoma in situ, are often poor surgical candidates. Imiquimod (IMQ), a toll-like receptor 7 agonist that activates innate immunity in the skin, is used to treat these cutaneous malignancies. However, IMQ's modest effect on the activation of adaptive immunity limits its efficacy as a monotherapy. In this study, we demonstrate that topical TSLP cytokine inducers—calcipotriol and retinoic acid—synergize with IMQ to activate CD4⁺ T-cell immunity against nonkeratinocyte cutaneous malignancies. Topical calcipotriol plus IMQ treatment reduced breast tumor growth compared with calcipotriol or IMQ alone (P < 0.0001). Calcipotriol plus IMQ–mediated tumor suppression was associated with significant infiltration of CD4⁺ effector T cells in the tumor microenvironment. Notably, topical calcipotriol plus IMQ immunotherapy enabled immune checkpoint blockade therapy to effectively control immunologically cold breast tumors, which was associated with induction of CD4⁺ T-cell immunity. Topical treatment with calcipotriol plus IMQ and retinoic acid plus IMQ also blocked subcutaneous melanoma growth. These findings highlight the synergistic effect of topical TSLP induction in combination with innate immune cell activation as an effective immunotherapy for malignancies affecting the skin.
The clinical effect of modified slow Mohs circular skin biopsy on penoscrotal extramammary Paget's disease
2023, Asian Journal of Surgery
Determining the tumor margin is the main problem in penoscrotal extramammary Paget's disease (PEMPD) surgery. This study aimed to explore the role of modified slow Mohs circular skin biopsy in determining the tumor margin in patients with PEMPD before operation.
Thirty-six PEMPD patients were divided into the control group and the observation group according to the simple randomization method. The control group, which consisted of 16 patients, underwent wide local excision, and the observation group, which consisted of 20 patients, underwent a modified slow Mohs circular skin biopsy to determine the tumor margin before surgery. The duration of surgery, number of frozen sections, satisfaction with the appearance of the skin, tumor recurrence rate, and incidence of functional sequelae were statistically analyzed.
The number of frozen sections, duration of surgery, incidence of functional sequelae, and tumor recurrence rate in the observation group were lower than in the control group. The satisfaction with the appearance of the skin was higher in the observation group than in the control group, and the difference was statistically significant (P < 0.05).
Modified slow Mohs circular skin biopsy can determine the tumor margin before surgery and reduce the number of frozen sections, tumor recurrence rate, and the incidence of functional sequelae. It also increases the postoperative satisfaction of patients.
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.
Sex differences in initial treatment for genital extramammary Paget disease in the United States: A systematic review
2023, Journal of the American Academy of Dermatology
Surgery is the standard treatment for genital extramammary Paget disease (gEMPD).
To determine if gEMPD treatments and outcomes differ by sex and US region.
A systematic review was performed of all English-language studies on initial gEMPD treatment in Medline via PubMed, Embase, Cochrane Library, Web of Science, and ClinicalTrials.gov. At least 2 reviewers performed title and abstract review and data extraction. Surgical categories included total skinning procedures, partial skinning procedures, Mohs micrographic surgery (MMS), or unspecified surgery. Chi-squared tests of association were used for comparisons.
A total of 60 studies comprising 302 (79.7%) female and 77 (20.3%) male patients met criteria. Women were most often initially recommended partial skinning procedures. In all, 74 (24.5%) women and 0 men underwent a total skinning procedure. Men were more likely to be offered MMS than women (40.3% vs 1.9%, P < .0001), despite men having a significantly greater extent of disease involving the perineum and perianal skin (21.1% vs 1.7%, P < .0001). Treatment recommendations varied by US region.
Only 20 states were represented.
Women are more frequently offered total skinning procedures for gEMPD while men are more frequently offered MMS. MMS offers less recurrence and morbidity than total skinning procedures and should be recommended equally to both female and male patients with gEMPD.

View all citing articles on Scopus

Funding sources: None.

Conflicts of interest: None identified.

View full text

Dermatologic SurgeryExtramammary Paget's disease: Surgical treatment with Mohs micrographic surgery

Background

Objective

Methods

Results

Conclusion

Section snippets

Methods

Results

Discussion

Urology

Am J Obstet Gynecol

J Am Acad Dermatol

Extramammary Paget's disease: a critical reexamination

Am J Dermatopathol

Carcinoma of moll's glands with extramammary Paget's disease of the eyelid

Cancer

Tumors of ceruminous glands

Cancer

Umbilical Paget's disease and prostatic carcinoma

Br J Dermatol

Long-term outcome of patients with perianal Paget's disease

Ann Surg Oncol

The Roswell Park Cancer Institute experience with extramammary Paget's disease

Br J Dermatol

Microscopically controlled surgery for extramammary Paget's disease

Arch Dermatol

Surgical treatment of extramammary Paget's disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision

Cancer

Dermatologic Surgery
Extramammary Paget's disease: Surgical treatment with Mohs micrographic surgery