Penoscrotal Extramammary Paget's Disease Research Articles

Superficial circumflex iliac artery perforator propeller flap for penoscrotal extramammary Paget's disease.

Extramammary Paget's disease (EMPD) is a rare intra-epidermal adenocarcinoma, and reconstruction of the penoscrotal region remains a clinical challenge. The superficial circumflex iliac artery perforator (SCIP) flap has been successfully used as a free flap, while its application as a propeller flap in the reconstruction of penoscrotal EMPD defect is rarely reported. The objective was to evaluate the safety and efficacy of the SCIP propeller flap in the reconstruction of penoscrotal defects in EMPD. Between September 2010 and August 2022, consecutive patients diagnosed with penoscrotal EMPD were enrolled. All patients underwent penoscrotal EMPD excision and reconstruction with SCIP propeller flap combined with other flaps or skin grafts on individual defects. Demographic information, surgical parameters, postoperative complications, patient satisfaction, and recurrence rates were analyzed. Twenty-four patients (mean age, 73 ± 8.8 years; mean BMI, 23.98 ± 3.62 kg/m2) with 33 SCIP propeller flaps (mean size 120.1 cm2; range, 24-208 cm2) were included, and the mean defect size was 67.4 cm2 (range, 12-255 cm2). The mean operative duration was 385.4 ± 146.8 min. Fifteen patients received autologous full-thickness skin grafts, and four received other flaps simultaneously. All flaps survived without total or partial loss, and all donor sites achieved primary closure, though local hematoma (one case) and limited skin graft necrosis (two cases) were observed. All patients experienced complete wound healing and maintained penoscrotal morphology and function without recurrent lesions over a mean follow-up of 59 months. The SCIP propeller flap seems a safe and effective reconstructive method for penoscrotal EMPD. IV.

Anatomic Subtype Differences in Extramammary Paget Disease

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. To compare demographic and tumor characteristics and treatment approaches for different EMPD subtypes. Recommendations for diagnosis and treatment are presented. MEDLINE, Embase, Web of Science Core Collection, and Cochrane Reviews CENTRAL from December 1, 1990, to October 24, 2022. 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. 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. 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). 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.

The clinical effect of modified slow Mohs circular skin biopsy on penoscrotal extramammary Paget's disease

ObjectiveDetermining 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. MethodsThirty-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. ResultsThe 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). ConclusionModified 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.

Multidisciplinary Approach for the Management of Penoscrotal Extramammary Paget's disease –An eUROGEN study

IntroductionWe reviewed the medical and surgical management and long-term outcomes for patients diagnosed with penoscrotal extramammary Pagets disease (EMPD) within an eUROGEN centre. Patients and MethodsRetrospective review of cases from an institutional database with biopsy proven penoscrotal EMPD. ResultsA 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. ConclusionReported 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.

Experiences with Surgical Reconstruction of Penoscrotal Extramammary Paget's Disease: A review of 21 cases

Penoscrotal extramammary Paget's disease is a rarely found intraepithelial adenocarcinoma in older male patients. The challenge for surgeons to perform high-quality post-surgery reconstruction is mostly case-dependent. Here, we present a review of post-surgical reconstruction practice in 21 cases using various scrotal skin flaps. During the 10-year study period (from 2008 to 2018), all 21 male patients diagnosed with penoscrotal extramammary Paget's diseases at Sun Yat-sen memorial hospital were enrolled in this study. All patients underwent expanding resections guided by frozen sections and penoscrotal reconstructions using various scrotal skin flaps alone or combined with pubic skin flaps and partial thickness skin graft depending on individual defects. The patient's clinical data, specifically the diagnosis, surgical outcome, and follow-up evaluation were retrospectively analyzed. The size of skin lesions ranged from 4 × 5 cm to 16 × 18 cm approximately. Out of 21 total cases, 13 patients received combined scrotal and pubic flaps, three patients received single scrotal flaps, and five patients received scrotal flaps and partial thickness skin grafts. Limited flap tip necrosis (two cases) and wound dehiscence (one case) were observed, and secondary resections were performed in four cases with positive post-operative pathological results. All patients experienced complete wound healing and had well-maintained penile morphology and function. Various scrotal skin flaps alone or combined with pubic skin flaps designed for individual lesion conditions in patients with penoscrotal extramammary Paget's disease are efficient for scrotal reconstruction. A good match of the local skin texture helps to maintain the morphology and function of the penis and scrotum.

Sequential somatic mutations upon secondary anti-HER2 treatment resistance in metastatic ERBB2S310F mutated extramammary Paget's disease.

Metastatic extramammary Paget’s disease is a rare adenocarcinoma with poor prognosis. Several reports of human epidermal growth factor receptor 2 alterations point to its pathogenic role in the disease. However, the occurrence of treatment resistance to anti-HER2 therapy demand the need for further knowledge. We report of a patient with metastatic penoscrotal extramammary Paget’s disease, with an ERBB2S310F mutation, in which near complete response was achieved upon treatment with trastuzumab and carboplatin. However, after 10 cycles of trastuzumab and carboplatin, widespread metastasis re-occurred. Analysis of a newly developing metastasis revealed additional genomic alterations including ERBB3A232V and PIK3CAG106V point mutations as well as MET and CDK6 amplification, providing a potential mechanism of acquired treatment resistance. Therefore, ERBB family inhibitor afatinib was initiated. Unfortunately, the patient succumbed to disease-related complications shortly after treatment initiation. This is the first report of ERBB2S310F mutated, metastatic extramammary Paget’s disease with secondary resistance to trastuzumab / carboplatin, potentially due to additional acquired genomic alterations. This case contributes to the growing evidence of HER2 in the pathogenesis of metastatic extramammary Paget’s disease and emphasizes the importance of repetitive, genomic analysis in rare diseases.

Human epidermal growth factor receptor 2 amplification as a biomarker for treatment in patients with lymph node-metastatic penoscrotal extramammary Paget's disease.

The role of human epidermal growth factor receptor 2 (HER2) amplification as a biomarker for treatment in patients with lymph node (LN)-metastatic penoscrotal extramammary Paget's disease (EMPD) was investigated in the present study. A total of 11 male patients with LN-metastatic penoscrotal EMPD were retrospectively reviewed. Positron emission tomography/computed tomography (PET/CT) was conducted prior to surgery. Immunohistochemistry and fluorescence in situ hybridization were used to evaluate HER2 gene amplification in LN samples. Sanger sequencing was used to investigate HER2 mutations. A literature review of the prevalence of HER2 amplification in EMPD and the efficacy of HER2-targeted therapy was also undertaken. PET/CT is effective in detecting metastatic sites. The sensitivity and specificity of PET/CT was 90.9 and 100.0% for inguinal LNs, and 85.7 and 80.0% for pelvic LNs, respectively. The median time from LN dissection to disease progression was 15.9±1.5 months. Of the 11 patients, 3 (27.3%) indicated HER2 amplification. Patients with HER2 amplification showed shorter median times from disease discovery to LN metastasis (HER2 amplification vs HER2 non-amplification; 15.6 vs. 10.0 months; P=0.50) and from LN dissection to disease progression (HER2 amplification vs. HER2 non-amplification, 16.2 vs. 13.6 months; P=0.11). However, the aforementioned observations were not indicated to be statistically significant. No HER2 mutations were identified. Trastuzumab, a HER2-targeted monoclonal antibody, was administered to 2 of the patients with HER2 amplification. A literature review of the prevalence of HER2 amplification in EMPD and the efficacy of HER2-targeted therapy showed similar results. Altogether, 485 cases of EMPD were reported, 35 of which had metastases. The results in the present study suggest that PET/CT should be used on all metastatic EMPD patients. EMPD may be effectively treated with trastuzumab. The present study and case reports from the literature provide evidence for the benefit of testing for HER2 amplification in this rare disease and highlight the requirement for a multicenter clinical trial to assess the impact of trastuzumab therapy in treating this disease.

Usefulness of Mapping Biopsy in the Treatment of Penoscrotal Extramammary Paget's Disease.

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy; however, the standard treatment of EMPD has not been established. In this study, we applied mapping biopsy to penoscrotal EMPD and evaluated its effects. A retrospective chart review was performed to determine the outcomes of patients with primary penoscrotal EMPD who underwent surgery at our institution between 2007 and 2014. Patients were divided into two groups (one group underwent mapping biopsy, while the other group did not), and the difference between the two groups was analyzed. The 5-year tumor-free rate was estimated using the Kaplan-Meier method, and the risk factors for local recurrence were also estimated. A total of 44 patients were analyzed, and the mean follow-up of patients was 50.27months. Patients who underwent mapping biopsy showed significantly lower tumor involvement at permanent and frozen biopsies and a lower local recurrence rate than those who did not undergo mapping biopsy. The 5-year tumor-free rate was significantly higher in the mapping biopsy group than in the non-mapping biopsy group. Multivariable analysis demonstrated that age at operation, mapping biopsy, and false-negative results at frozen biopsy were associated with local recurrence. Mapping biopsy is beneficial to reduce local recurrence in penoscrotal EMPD.

Mammary and Extramammary Paget's Disease Presented Different Expression Pattern of Steroid Hormone Receptors.

Background and Objectives Paget's disease (PD) is a rare intraepithelial adenocarcinoma, which is composed of mammary (MPD) and extramammary Paget's disease (EMPD). Currently, the published literature contains scant data on expression pattern of steroid hormone receptors in MPD and EMPD. Methods Expression of estrogen receptor (ER) and androgen receptor (AR) was evaluated in 88 MPD and 72 EMPD by using immunohistochemical staining and H-score method. Results Positive expression of AR was significantly higher in EMPD (61.11%, 44/72) than in MPD (32.95%, 29/88) (P < 0.001), while ER expression was positive 19.44% (14/72) in EMPD and only 9.09% (8/88) in MPD (P = 0.059). ER-AR expression pattern was significantly different between MPD (3.41%, 3/88) and EMPD (16.67%, 12/72) (P < 0.001). No difference of AR (P = 0.301) or ER (P = 0.239) expression was identified between invasive (48.57%, 51/105 of AR, and 11.43%, 12/105 of ER) and noninvasive PD. In MPD, no difference of AR expression between MPD alone (7/18, 38.89%) and MPD with underling ductal carcinoma of breast (22/70, 31.43%) was identified (P = 0.548). In EMPD, expression of AR was 63.33% (38/60) in penoscrotal EMPD. Conclusion Our current results indicate that MPD and EMPD presented different expression pattern of AR and ER and would help to further identify the molecular subtype of MPD and EMPD for adjuvant hormonal therapy, especially for patients with penoscrotal EMPD.

Primary invasive carcinoma associated with penoscrotal extramammary Paget's disease: a clinicopathological analysis of 56 cases.

To investigate the clinicopathological features, therapeutic strategies, and prognostic factors of patients with penoscrotal invasive extramammary Paget's disease (EMPD). We retrospectively collected clinical, pathological, and follow-up data of 56 men with invasive penoscrotal EMPD. Histopathological features of the primary skin lesion including tumour size, surgical margin status, depth of invasion and lymphovascular invasion were examined. The median age was 67 years and median longest diameter of lesion was 5 cm. All patients were treated with wide surgical excision and 22 patients with clinically positive regional lymph nodes underwent therapeutic regional lymph node dissection. At the end of the study, 44.6% of patients developed distant metastasis and 39.3% of patients had died from disease. Univariate analysis showed that patients with one of the following poor prognostic factors: depth of invasion of lower dermis or deeper, presence of lymphovascular invasion and regional lymph node metastasis at diagnosis, had significantly shorter cancer-specific survival time. Multivariate analysis found that depth of invasion was the only independent prognostic factor. The prognosis of invasive EMPD is significantly associated with depth of invasion, lymphovascular invasion and regional lymph node status. More aggressive therapy and more rigorous follow-up should be recommended for patients with these poor prognostic factors.

Penoscrotal Extramammary Paget's Disease with Multiple Lymph Node Metastasis

Penoscrotal Extramammary Paget's Disease with Multiple Lymph Node Metastasis

Penoscrotal extramammary Paget's disease: surgical techniques and follow-up experiences with thirty patients

To report the surgical management, complications and prognosis of patients with penoscrotal extramammary Paget's disease (EMPD) at different clinical stages. Between 2003 and 2008, a total of 30 male patients with penoscrotal EMPD were enrolled and evaluated. All enrolled subjects received frozen biopsy-guided local wide resection and immediate reconstruction. Patients were followed every 3 months postoperatively. Among the 30 patients who accepted and underwent frozen biopsy-guided local wide resection treatment and reconstruction, two (6.7%) cases exhibited positive margins, verified by pathological examination, and underwent re-excision after surgery. The technique of primary closure or an adjacent flap was used in 10 (33.3%) cases, split-thickness skin grafts were used in 15 (50%), and an anterolateral thigh perforator flap was used in five cases (16.7%). The postoperative complications were acceptable. The mean follow-up time was 64.9 ± 29.6 months. Of all 30 cases, 22 patients (73.3%) survived with no evidence of recurrence, four patients (13.3%) exhibited local recurrence, two patients (6.7%) exhibited both local recurrence and distant metastasis and the remaining two patients (6.7%) exhibited distant metastasis. Five patients died from metastasis or cachexia. Current surgical techniques, including primary closure, adjacent flaps, split-thickness skin flaps and anterolateral thigh perforator flaps are able to reconstruct all types of defects with acceptable complications. Some patients with negative margins went on to exhibit local recurrence, potentially due to adnexal carcinoma or internal malignancy.

Reconstruction with scrotal skin flaps after wide local resection of penoscrotal extramammary Paget's disease

Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? Both the treatment and reconstruction after resection of penoscrotal extramammary Paget's disease (EMPD) are of concern among oncological urologists. Reconstruction of large skin defects after wide local resection of this disease remains challenging and skilled work. For the first time, by using scrotal skin flaps, we have introduced an effective and reliable procedure into the new area of reconstruction for penoscrotal EMPD in selected patients, with satisfactory functional and aesthetic results. • To introduce an effective procedure for reconstruction of large skin defects after wide local resection of penoscrotal extramammary Paget's disease (EMPD) in selected patients using scrotal skin flaps without compromising oncological control. • From January to June 2010 this procedure was carried out in five selected patients with penoscrotal EMPD who underwent wide local resection with subsequent reconstruction using scrotal skin flaps. • Patient demographics, lesion characteristics, surgical margins and outcomes were evaluated. • Skin defects covered 48-96 cm(2) (median = 72 cm(2)). The median (range) duration of follow-up was 24 (22-27) months. • Flaps survived with satisfactory functional and aesthetic results. • All patients survived without local or systemic recurrence of tumour. • Wide local resection can provide cure in superficial penoscrotal EMPD. • The described method using scrotal skin flaps seems to be a safe and effective procedure for the reconstruction of large defects.

Penoscrotal Extramammary Paget's Disease: The University of Texas M. D. Anderson Cancer Center Contemporary Experience

Extramammary Paget's disease is a rare intra-epithelial malignancy that is occasionally associated with an invasive adenocarcinoma component as well as other secondary cancers. We investigated a consecutive series of patients referred for extramammary Paget's disease of the penis or scrotum at a single center in the contemporary era to determine the presence of secondary cancers and treatment outcomes. Between December 1990 and February 2009, 20 patients with extramammary Paget's disease of the scrotum and/or penis were seen. Patients were investigated with computerized tomography of the abdomen/pelvis, chest x-ray, cystoscopy, colonoscopy and serum prostate specific antigen measurement to rule out obvious secondary cancer. Clinical charts were reviewed with institutional review board approval. Mean followup of treated cases was 48 months (range 7 to 208). Patients were 50 to 86 years old with a history of symptoms of between 6 months and 10 years. Eight patients had invasive adenocarcinoma, of whom 2 died of the disease and 3 who had disease at 5, 7 and 40 months, respectively, were undergoing multimodal therapy. No patient with confirmed intra-epidermal disease only died of the disease. Invasive disease predicted nodal and metastatic progression. Surgical resection to achieve tumor-free margins resulted in durable relapse-free survival of patients with intra-epidermal extramammary Paget's disease alone. Extramammary Paget's disease with invasion was associated with regional metastatic progression. The latter scenario as well as failure to treat localized extramammary Paget's disease alone was associated with a fatal outcome. Systemic chemotherapy should be further explored in patients with invasive adenocarcinoma or lymph node positive disease.

P74 Resurfacing the penoscrotum with local pedicle flaps at large defects after ablation of penoscrotal extramammary Paget's disease

Purpose Penoscrotal extramammary Paget's disease is a rare cutaneous malignancy that primarily affects the elderly. To prevent local recurrence, adequate surgical excision with an intraoperative frozen section, proper reconstruction, and a long-term period of follow-up are required. The present study describes the treatment of patients with penoscrotal extramammary Paget's disease, with a focus on post-oncologic reconstruction. Methods Eleven patients who had undergone a local pedicle flap procedure due to the large body defects after ablation of extramammary Paget's disease of the penoscrotal area, during the period of 1999–2009. Local wide excision with intraoperative frozen sectioning was performed, and the penoscrotal wound was reconstructed with a local skin flap. Various flaps were chosen depending on the site and the defected size. If the defect size was small and scrotal tissue was adequate, only a scrotal flap (n=5) was completed for the reconstruction. Large defects with insufficient remnant scrotal tissue as well as cases in which the pubic or inguinal area was involved went through a groin flap (n=2) or an anterolateral thigh flap (n=4) among the local pedicle flaps. Results There were no complications with the postoperative wound. Furthermore, no local recurrence was noted during two to six years of follow-up (mean average 3.5 years). Conclusion For the resurfacing the penoscrotum at large defects after ablation of extramammary Paget's disease, we performed reconstruction with a local pedicle flap. In cases where the remnant tissue was sufficient, a scrotum flap was primarily used, and if additional tissue was needed a local pedicle flap; groin flap or anterolateral thigh flap, etc was applied for reconstruction. In terms of both function and cosmetic concerns, the results were satisfactory. Key Words: Penoscrotal extramammary Paget's disease, Scrotal flap, Groin flap, Anterolateral thigh flap

Rotary mutiflaps for defect reconstruction in penoscrotal extramammary Paget's disease

Purpose To describe a method of using rotary mutiflaps from scrotum, penis, and mons pubis to reconstruct large skin defect after local wide excision of penoscrotal extramammary Paget's disease (EMPD). Materials and methods We reviewed our databases for the period between 1997 and 2005, and identified 5 cases of penoscrotal EMPD, all male aged between 56 to 69 (mean 63.2 years), who underwent local wild excision with subsequent reconstruction by rotary multiflaps. Results All flaps survived with satisfactory functional and aesthetic results. No signs of recurrent disease or metastasis occurred during the follow-up period ranging from 5 to 36 months (mean 20 months). Conclusions Rotary mutiflaps is a safe and effective procedure for the reconstruction of large defect in penoscrotal EMPD.

Penoscrotal extramammary Paget's disease: a review of 33 cases in a 20-year experience.

Extramammary Paget's disease in men most frequently involves the penoscrotal area. The uncertainty of the outcome and of the relationship to the underlying adnexal carcinoma and associated internal malignancy still exists. From 1982 to 2001, 33 patients with penoscrotal extramammary Paget's disease were treated and followed up. Therapeutic modalities included carbon dioxide laser ablation (two patients) and local wide excision (31 patients). Split-thickness skin graft (22 patients), local scrotal flap (six patients), and primary closure (three patients) were utilized to reconstruct the penoscrotal defects after local wide excision. An underlying adnexal carcinoma occurred in seven of 33 patients (21.2 percent). The incidence of associated internal malignancy was 9.1 percent (three of 33 patients), including one concurrently and two nonconcurrently associated malignancies. Eight of 33 patients had local recurrence, representing an incidence of 24.2 percent. Three patients (9.1 percent) had distant metastasis and ultimately died of metastatic carcinoma. Of these patients, 31 were grouped according to the degrees of involvement: limited to the epidermis (group 1, n = 14), involvement of the adnexal gland and/or hair follicle (group 2, n = 10), and the presence of an underlying adnexal carcinoma (group 3, n = 7). Local wide excision with subsequent reconstruction by split-thickness skin graft was favored in this series. Patients with an underlying adnexal carcinoma or pathological invasion of the dermis (group 2 or 3) had a worse prognosis than patients without. From this study, it is difficult to address the particular relationship between the outcome and the associated internal malignancy.

EXTRAMAMMARY PAGET's DISEASE OF THE PENIS AND SCROTUM: EXCISION, RECONSTRUCTION AND EVALUATION OF OCCULT MALIGNANCY

We describe treatment and reconstruction in patients after surgery for extramammary Paget's disease of the penis and scrotum. We also investigated whether this disease causes an increased risk of undiagnosed visceral malignancy. We reviewed the databases at our institution from 1996 to 2000 and identified 6 men 67 to 87 years old (mean age 76). In addition, we reviewed the literature on the clinical and pathological features of this disease. In our 6 patients scrotal involvement was present in 83% and penile extramammary Paget's disease was present in 33%. Each man underwent wide local excision and large skin defects were immediately reconstructed with split-thickness skin grafts. In 1 case extramammary Paget's disease had spread to the superficial inguinal nodes. At a mean followup of 29 months there has been no local recurrence and internal malignancy has not been diagnosed. Our literature review revealed 13 patients with penoscrotal extramammary Paget's disease and visceral malignancy, including 12 (92%) with malignancy of the genitourinary system. Extramammary Paget's disease of the penis and scrotum is a rare disease that can be managed by excision and immediate reconstruction with skin grafting or a local skin flap. Disease may spread to the regional lymph nodes. Although genitourinary cancer may accompany penoscrotal extramammary Paget's disease, an extensive search for cancer of the thorax or abdomen may be unnecessary because only 1 reported case of colon cancer has been associated with penile or scrotal extramammary Paget's disease.