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1.
Pathol Oncol Res ; 30: 1611803, 2024.
Article in English | MEDLINE | ID: mdl-38978790

ABSTRACT

We have developed a biopsy technique aimed at preoperative evaluating the extent of Paget's vulvar disease in order to plan subsequent radical vulvar surgery. The aim is to find all possible lesion sites that are not visible macroscopically, to obtain a clear evaluation of the disease spread and to tailor the radical surgical procedure to remove even microscopic lesions, avoiding recurrences and excessively destructive surgery, adopting as conservative an approach as possible. We used this procedure for the first time to establish the radicality of the surgical intervention in a 68-year-old patient initially suffering from a single invasive vulvar Paget's lesion.


Subject(s)
Paget Disease, Extramammary , Vulvar Neoplasms , Humans , Female , Aged , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Vulvar Neoplasms/surgery , Vulvar Neoplasms/pathology , Biopsy/methods , Preoperative Care/methods
2.
J Dermatolog Treat ; 35(1): 2368066, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38897607

ABSTRACT

PURPOSE: To evaluate the efficacy of Mohs micrographic surgery (MMS) combined with photodynamic therapy (PDT) in treating non-invasive extramammary Paget's disease (EMPD). MATERIALS AND METHODS: A 77-year-old male patient with non-invasive EMPD was treated with MMS followed by PDT. Preoperative fluorescence localization using 5-aminolevulinic acid (ALA) was performed to determine the surgical scope. MMS was conducted under lumbar anesthesia with intraoperative frozen-section pathology. Postoperative PDT was administered weekly for three sessions. RESULTS: The patient achieved negative surgical margins after two rounds of intraoperative pathology. Postoperative follow-up over two years showed no recurrence, and the patient did not experience significant adverse reactions. CONCLUSION: The combination of MMS and PDT was effective in treating non-invasive EMPD, demonstrating favorable clinical outcomes and no recurrence over the two-year follow-up period.


Subject(s)
Aminolevulinic Acid , Mohs Surgery , Paget Disease, Extramammary , Photochemotherapy , Photosensitizing Agents , Skin Neoplasms , Humans , Male , Aged , Paget Disease, Extramammary/pathology , Paget Disease, Extramammary/drug therapy , Paget Disease, Extramammary/surgery , Aminolevulinic Acid/therapeutic use , Skin Neoplasms/pathology , Skin Neoplasms/drug therapy , Skin Neoplasms/surgery , Skin Neoplasms/therapy , Photosensitizing Agents/therapeutic use , Treatment Outcome , Combined Modality Therapy , Margins of Excision
3.
Actas Dermosifiliogr ; 115(6): T555-T571, 2024 Jun.
Article in English, Spanish | MEDLINE | ID: mdl-38648936

ABSTRACT

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.


Subject(s)
Carcinoma, Basal Cell , Margins of Excision , Mohs Surgery , Paraffin Embedding , Skin Neoplasms , Humans , Skin Neoplasms/surgery , Skin Neoplasms/pathology , Carcinoma, Basal Cell/surgery , Carcinoma, Basal Cell/pathology , Carcinoma, Squamous Cell/surgery , Carcinoma, Squamous Cell/pathology , Neoplasm Recurrence, Local/prevention & control , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Hutchinson's Melanotic Freckle/surgery , Hutchinson's Melanotic Freckle/pathology , Dermatofibrosarcoma/surgery , Dermatofibrosarcoma/pathology
5.
JAMA Dermatol ; 160(4): 417-424, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38446447

ABSTRACT

Importance: Extramammary Paget disease (EMPD) is a rare, highly recurrent cutaneous malignant neoplasm of unclear origin. EMPD arises most commonly on the vulvar and penoscrotal skin. It is not presently known how anatomic subtype of EMPD affects disease presentation and management. Objective: To compare demographic and tumor characteristics and treatment approaches for different EMPD subtypes. Recommendations for diagnosis and treatment are presented. Data Sources: MEDLINE, Embase, Web of Science Core Collection, and Cochrane Reviews CENTRAL from December 1, 1990, to October 24, 2022. Study Selection: Articles were excluded if they were not in English, reported fewer than 3 patients, did not specify information by anatomic subtype, or contained no case-level data. Metastatic cases on presentation were also excluded. Data Extraction and Synthesis: Abstracts of 1295 eligible articles were independently reviewed by 5 coauthors, and 135 articles retained. Reporting was in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines. The analysis was cunducted in August 2019 and updated in November 2022. Findings: Most vulvar EMPD cases were asymptomatic, and diagnosis was relatively delayed (mean, 25.1 months). Although most vulvar EMPD cases were intraepidermal (1247/1773 [70.3%]), radical surgeries were still performed in almost one-third of cases. Despite this aggressive surgical approach, 481 of 1423 (34%) recurred, commonly confined to the skin and mucosa (177/198 [89.4%]). By contrast, 152 of 1101 penoscrotal EMPD cases (14%) recurred, but more than one-third of these recurrences were regional or associated with distant metastases (54 of 152 [35.5%]). Perianal EMPD cases recurred in one-third of cases (74/218 [33.9%]), with one-third of these recurrences being regional or associated with distant metastasis (20 of 74 [27.0%]). Perianal EMPD also had the highest rate of invasive disease (50% of cases). Conclusions and Relevance: The diagnosis and treatment of EMPD should differ based on anatomic subtypes. Considerations for updated practice may include less morbid treatments for vulvar EMPD, which is primarily epidermal, and close surveillance for local recurrence in vulvar EMPD and metastatic recurrence in perianal EMPD. Recurrences in penoscrotal subtype were less common, and selective surveillance in this subtype may be considered. Limitations of this study include the lack of replication cohorts and the exclusion of studies that did not stratify outcomes by anatomic subtype.


Subject(s)
Paget Disease, Extramammary , Female , Humans , Paget Disease, Extramammary/diagnosis , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Perineum/pathology , Vulva/pathology
6.
Medicine (Baltimore) ; 103(13): e37541, 2024 Mar 29.
Article in English | MEDLINE | ID: mdl-38552095

ABSTRACT

BACKGROUND: Extramammary Paget disease is a relatively rare and less malignant intraepithelial adenocarcinoma. t is found in areas with abundant distribution of apocrine sweat glands such as the external genitalia, external genitalia, and perianal area, with fewer armpits. The disease progresses slowly and is prone to misdiagnosis in clinical practice. METHODS: We retrospectively analyzed a female patient. She had a left axillary mass for more than 2 years. Recently, the mass increased and the surface skin was ulcerated. Then she went to Jiangxi Provincial Dermatology Hospital for left axillary lesion resection, and the postoperative pathology showed Paget disease outside the breast. For further diagnosis and treatment, she came to our hospital. We diagnosed a tumor with uncertain or unknown dynamics in the left axillary breast. Under general anesthesia, left subaxillary mass resection, freezing and left breast cancer breast conserving surgery was performed. RESULTS: The postoperative pathology of the left axillary mass combined with morphological and immunohistochemical results was consistent with Paget disease. Postoperative immunohistochemistry showed estrogen receptor (+, 20%), progesterone receptor (-), human epidermal growth factor receptor-2 (3+), Ki-67 (30%), cytokine7 (+), and p63 (-). Following up for 22 months, there has been no local recurrence, no swelling of the right axillary lymph node, no distant metastasis found on follow-up, and no complications such as upper limb lymphedema, upper limb sensory abnormalities, or motor disorders have been observed. CONCLUSION: Paget disease outside the axillary breast is relatively rare, and surgical resection is the best choice. The prognosis is good, and the recurrence rate is low.


Subject(s)
Adenocarcinoma , Breast Neoplasms , Osteitis Deformans , Paget Disease, Extramammary , Humans , Female , Retrospective Studies , Adenocarcinoma/surgery , Paget Disease, Extramammary/diagnosis , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Lymph Nodes/pathology , Breast , Breast Neoplasms/diagnosis , Breast Neoplasms/surgery , Breast Neoplasms/pathology
8.
Urol Int ; 108(3): 264-271, 2024.
Article in English | MEDLINE | ID: mdl-38368870

ABSTRACT

INTRODUCTION: Extramammary Paget's disease of the scrotum and penis is a relatively rare cutaneous malignant tumor. At present, its pathogenesis, and clinical and pathological characteristics are not very clear. This is controversial regarding surgical margin width to decrease the high recurrence rate. This paper aimed to report the case and review the literature of extramammary Paget's disease of scrotum and penis. CASE PRESENTATION: We presented the case of a 74-year-old male patient with the patchy erythema and pruritus in the perineum who was admitted to our department. Biopsy of the large plaque revealed Paget disease. Under the condition of ensuring negative surgical margins by rapid frozen pathology, a wide local excision of the lesion, bilateral orchiectomy, and adnexectomy were performed on the patient. Pathology revealed that many scattered vacuolated Paget cells were observed in the epidermal layer, and the diagnosis was Paget's disease of the scrotum and penis. The 2 cm outside the skin lesion was used as the initial surgical margin, and free skin flap transplantation was used to repair the surgical wound. The patient recovered well and was discharged 1 week after surgery. CONCLUSION: Currently, histopathologic biopsy is the most important diagnostic method for EMPD. Once confirmed, for patients eligible for surgical intervention, wide local excision of the lesion and rapid intraoperative frozen pathological examination should be performed as soon as possible. The skin flap transplantation is the first choice for the repair of large-scale wound after surgery.


Subject(s)
Paget Disease, Extramammary , Penile Neoplasms , Scrotum , Humans , Male , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Paget Disease, Extramammary/diagnosis , Scrotum/pathology , Scrotum/surgery , Aged , Penile Neoplasms/surgery , Penile Neoplasms/pathology , Genital Neoplasms, Male/surgery , Genital Neoplasms, Male/pathology , Genital Neoplasms, Male/diagnosis , Biopsy , Treatment Outcome
9.
Actas Dermosifiliogr ; 115(6): 555-571, 2024 Jun.
Article in English, Spanish | MEDLINE | ID: mdl-38395222

ABSTRACT

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.


Subject(s)
Carcinoma, Basal Cell , Margins of Excision , Mohs Surgery , Paraffin Embedding , Skin Neoplasms , Humans , Skin Neoplasms/surgery , Skin Neoplasms/pathology , Carcinoma, Basal Cell/surgery , Carcinoma, Basal Cell/pathology , Carcinoma, Squamous Cell/surgery , Carcinoma, Squamous Cell/pathology , Neoplasm Recurrence, Local/prevention & control , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Hutchinson's Melanotic Freckle/surgery , Hutchinson's Melanotic Freckle/pathology , Dermatofibrosarcoma/surgery , Dermatofibrosarcoma/pathology
12.
J Am Acad Dermatol ; 90(1): 66-73, 2024 01.
Article in English | MEDLINE | ID: mdl-37704106

ABSTRACT

BACKGROUND: Evidence regarding long-term therapeutic outcomes and disease-specific survival (DSS) in Extramammary Paget's disease (EMPD) is limited. OBJECTIVES: To assess the DSS and outcomes of surgical and nonsurgical therapeutic modalities in a large cohort of EMPD patients. METHODS: Retrospective chart review of EMPD patients from 20 Spanish tertiary care hospitals. RESULTS: Data on 249 patients with a median follow-up of 60 months were analyzed. The estimated 5-, 10-, and 15-year DSS was 95.9%, 92.9%, and 88.5%, respectively. A significantly lower DSS was observed in patients showing deep dermal invasion (≥1 mm) or metastatic disease (P < .05). A ≥50% reduction in EMPD lesion size was achieved in 100% and 75.3% of patients treated with surgery and topical therapies, respectively. Tumor-free resection margins were obtained in 42.4% of the patients after wide local excision (WLE). The 5-year recurrence-free survival after Mohs micrographic surgery (MMS), WLE with tumor-free margins, WLE with positive margins, radiotherapy, and topical treatments was 63.0%, 51.4%, 20.4%, 30.1%, and 20.8%, respectively. LIMITATIONS: Retrospective design. CONCLUSIONS: EMPD is usually a chronic condition with favorable prognosis. MMS represents the therapeutic alternative with the greatest efficacy for the disease. Recurrence rates in patients with positive margins after WLE are similar to the ones observed in patients treated with topical agents.


Subject(s)
Paget Disease, Extramammary , Humans , Retrospective Studies , Paget Disease, Extramammary/surgery , Mohs Surgery , Survival Analysis , Margins of Excision , Treatment Outcome , Neoplasm Recurrence, Local/epidemiology , Neoplasm Recurrence, Local/therapy , Neoplasm Recurrence, Local/pathology
13.
PLoS One ; 18(11): e0294152, 2023.
Article in English | MEDLINE | ID: mdl-37956192

ABSTRACT

INTRODUCTION: Perineal extramammary paget's disease (EMPD) is characterized with high recurrence rate. Although numerous therapeutic measures for this disease have been reported so far, it is unknown whether there is significant difference in their recurrence-preventing efficiency. This study aims to compare the recurrence outcomes of reported perineal EMPD treatments. METHODS: We searched public databases of for published studies concerning perineal EMPD treatments. After screening by inclusion and exclusion criteria, we extracted the data relevant to recurrence rate, and conducted network meta-analysis (NMA) by using Bayesian random-effects approach. RESULTS: Our analysis included 29 previous studies (involving both male and female patients) and 11 treatment designs which are wide local excision (WLE), local excision (LE), Mohs micrographic surgery (MMS), radiotherapy (RT), radical vulvectomy (RV), photodynamic therapy (PDT), lasers (LS), imiquimod, and WLE+RT, WLE+PDT, WLE+LS. Comparing with WLE, the MMS showed significant advantage in reducing recurrence [OR: 0.18 (0.03-0.87)], while none of the rest treatments has statistically significant results. After removing outlier studies, MMS still has the significant advantages [OR: 0.35 (0.11-0.82)], and LE turned to be the treatment with worst performance [OR: 13 (2.50-110)]. Covariance analysis of follow-up length, gender differences, and lesion locations indicated only short follow-up time could affect the recurrence statistics, which tend to conceal the real differences. Funnel plot demonstrated there is no significant small study effect. CONCLUSION: MMS has the best performance on reducing perineal EMPD recurrence, while LE exhibits the worst capability in such regard. Recurrence-preventing abilities of other treatments have no significant difference between each other.


Subject(s)
Paget Disease, Extramammary , Humans , Male , Female , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Bayes Theorem , Network Meta-Analysis , Mohs Surgery/methods , Imiquimod , Neoplasm Recurrence, Local/pathology , Retrospective Studies
14.
In Vivo ; 37(6): 2618-2627, 2023.
Article in English | MEDLINE | ID: mdl-37905666

ABSTRACT

BACKGROUND/AIM: Data regarding the clinicopathological factors predicting recurrence and prognosis in patients with vulvar extramammary Paget disease (VPD) are limited. Therefore, we aimed to identify predictive factors for recurrence and outcomes in patients with VPD. PATIENTS AND METHODS: Forty-five patients with VPD were included in this study. We reviewed electronic medical records and pathology slides to collect clinicopathological information. RESULTS: Eighteen cases (40.0%) had resection margin (RM) involvement. Twelve patients (26.7%) received adjuvant radiation therapy (RT). Ten patients (22.2%) experienced recurrence. The recurrence rate was higher in patients who underwent wide local excision or simple vulvectomy than in those who underwent radical vulvectomy. Positive RM involvement was a significant and independent predictive factor for worse recurrence-free survival (RFS). The overall survival rate of patients who received adjuvant RT was significantly higher than that of those who underwent surgery alone. CONCLUSION: A positive RM involvement independently predicted worse RFS. The recurrence rate was significantly associated with the type of surgical procedure performed. Additionally, adjuvant RT can improve the prognosis of patients with VPD.


Subject(s)
Paget Disease, Extramammary , Vulvar Neoplasms , Female , Humans , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Vulvar Neoplasms/pathology , Prognosis , Gynecologic Surgical Procedures , Margins of Excision , Neoplasm Recurrence, Local/pathology , Retrospective Studies
15.
In Vivo ; 37(6): 2786-2791, 2023.
Article in English | MEDLINE | ID: mdl-37905610

ABSTRACT

BACKGROUND/AIM: Extramammary Paget's disease (EMPD) is a rare, slow growing intra-epidermal malignant neoplasm that arises in areas rich in apocrine glands. Several common sites of occurrence have been reported, including the vulva, perianal region, perineum, and scrotum. Most relevant studies rely on small data bases. Our objective was to evaluate prognostic factors of EMPD patients at a single medical center. PATIENTS AND METHODS: We retrospectively analyzed 19 patients (8 males, 11 females) diagnosed with genital EMPD who were treated at the Taichung Veterans General Hospital between 2006/04 and 2022/08. Collected information included tumor location, margin condition in the case of surgical resection, recurrence rate, recurrence management, accompanied gastrointestinal malignancy, treatment details and survival data. RESULTS: Among 19 cases, 4 with initial margin being positive, and 3 received second surgery (one refused surgery and another expired within a year). Tumor recurrence was found in 7 cases, with 6 of them later receiving second surgery, and the remaining one received radiation therapy. Median DFS was 7.57 years. During the 15-year follow-up, 2 patients expired. Overall survival rate was 87.5%. Among all factors we had analyzed, only those accompanied with GI tract malignancy had significantly worse survival rate (p=0.018). Frozen sections taken at surgical margin during surgery significantly reduced cancer recurrence rate (p=0.45). Permanent pathology margins appeared to affect the recurrence rate, but that was not significant when comparing with intraoperative frozen sections. CONCLUSION: Local wide excision with skin flap reconstruction remains the major treatment option for genital EMPD. Following the standard-of-care procedure, the overall patient outcome was excellent. Among factors potentially associated with recurrence rate, intraoperative frozen biopsy was the most significant one. Performing intraoperative frozen biopsy is essential for recurrence-free rate elevation.


Subject(s)
Paget Disease, Extramammary , Male , Female , Humans , Retrospective Studies , Paget Disease, Extramammary/diagnosis , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Prognosis , Taiwan/epidemiology , Neoplasm Recurrence, Local
17.
J Gynecol Oncol ; 34(6): e76, 2023 11.
Article in English | MEDLINE | ID: mdl-37477099

ABSTRACT

OBJECTIVE: Extramammary Paget's disease (EMPD) of the vulva is a rare disease which predominantly presents in postmenopausal Caucasian women. As yet, no studies on Asian female patients with EMPD have been performed. This study aimed to identify the clinical features of patients with vulvar EMPD in Korea, and to evaluate the risk factors of recurrence and postoperative complications in surgically treated EMPD. METHODS: We retrospectively reviewed 47 patients with vulvar EMPD who underwent wide local excision or radical vulvectomy. The clinical data and surgical and oncological outcomes following surgery were extracted from medical records and analyzed. Univariate and multivariate analyses for predicting recurrence and postoperative complications were performed. RESULTS: 21.3% of patients had complications after surgery, and wound dehiscence was the most common. 14.9% of patients experienced recurrence, and the median interval to recurrence from initial treatment was 69 (range 33-169) months. Vulvar lesions larger than 40 mm was the independent risk factor of postoperative complications (odds ratio [OR]=7.259; 95% confidence interval [CI]=1.545-34.100; p=0.012). Surgical margin status was not associated with recurrence in surgically treated vulvar EMPD patients (OR=0.83; 95% CI=0.16-4.19; p=1.000). CONCLUSION: Positive surgical margin is a frequent finding in the patients with vulvar EMPD, but disease recurrence is not related with surgical margin status. Since EMPD is a slow growing tumor, a surveillance period longer than 5 years is required.


Subject(s)
Paget Disease, Extramammary , Vulvar Neoplasms , Humans , Female , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Prognosis , Retrospective Studies , Margins of Excision , Neoplasm Recurrence, Local/epidemiology , Neoplasm Recurrence, Local/pathology , Vulva/surgery , Vulva/pathology , Vulvar Neoplasms/surgery , Vulvar Neoplasms/pathology , Postoperative Complications/epidemiology
18.
J Plast Reconstr Aesthet Surg ; 84: 392-397, 2023 09.
Article in English | MEDLINE | ID: mdl-37399659

ABSTRACT

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.


Subject(s)
Margins of Excision , Paget Disease, Extramammary , Female , Humans , Male , Biopsy , Paget Disease, Extramammary/ethnology , Paget Disease, Extramammary/pathology , Paget Disease, Extramammary/surgery , Retrospective Studies , Singapore , Asia, Southeastern
19.
Arch Dermatol Res ; 315(9): 2513-2518, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37266674

ABSTRACT

Sentinel lymph node biopsy is increasingly used to detect subclinical nodal metastases in extramammary Paget disease. We performed a comprehensive systematic review of the literature to further explore the role of sentinel lymph node biopsy in extramammary Paget disease. Five databases were searched for relevant terms. Articles were included if they were in English and presented primary data on at least one patient with extramammary Paget disease who underwent sentinel lymph node biopsy in the absence of lymphadenopathy or known metastatic disease. Twenty-eight articles were included, with 366 subjects. Seventy-seven sentinel node biopsies (21.2%) were positive, including 12 in which the primary tumor had microinvasion (15.6%) and 56 with deep invasion (72.7%). Of the positive cases, 11 (14.3%) had no further treatment, 54 (70.1%) underwent nodal dissection, 4 (5.2%) were treated with systemic agents, and 1 (1.3%) had radiation. After a mean follow up of 24 months, 9 subjects with a positive lymph node biopsy experienced nodal recurrence (11.7%), 15 had distant metastases (19.5%), and 13 died of the disease (16.9%). In conclusion, invasive extramammary Paget disease is strongly associated with poor outcomes including nodal metastasis, distant metastasis, and disease specific death. Sentinel lymph node biopsy is a useful tool to screen for subclinical nodal metastases in invasive disease, and can be used to help guide clinical management.


Subject(s)
Paget Disease, Extramammary , Skin Neoplasms , Humans , Sentinel Lymph Node Biopsy , Lymphatic Metastasis , Paget Disease, Extramammary/diagnosis , Paget Disease, Extramammary/surgery , Paget Disease, Extramammary/pathology , Skin Neoplasms/pathology
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