Extramammary Paget disease (EMPD) is a rare dermatologic condition that frequently presents in areas where apocrine sweat glands are abundant, most commonly the vulva, although perineal, scrotal, perianal, and penile skin may also be affected. Lesions clinically present as erythematous, well-demarcated plaques that may become erosive, ulcerated, scaly, or eczematous. Extramammary Paget disease has a female predominance and usually occurs in the sixth to eighth decades of life. Professionals disagree about many aspects of EMPD, for example, the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. Early recognition is imperative because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease.
Park et al. report their experience in the management of extramammary Paget’s Disease (EMPD) of the penoscrotal region and specifically compare outcomes among cohorts of men with the disease who either did or did not undergo mapping biopsies prior to their definitive surgical procedure. The rationale for the study and this comparison is that Paget’s disease initially spreads insidiously through the epidermis, sometimes in a single-cell fashion, and establishing the diagnosis can be very difficult subsequent to intraoperative frozen sections. Thus, several studies have described the use of outpatient mapping biopsies under more permanent section pathology techniques to facilitate the diagnosis and to ‘clear’ the surgical margins (references 19–21 in the article). This should theoretically lead to a lower incidence of positive frozen section margins intraoperatively, a lower incidence of positive permanent section margins, and lower recurrence rates for patients.
Primary invasive Extramammary Paget’s vulvar disease is a rare tumor that is challenging to control. Wide surgical excision represents the standard treatment approach for Primary invasive Extramammary Paget’s vulvar disease. The goal of the current study was to analyze the appropriate indications of radiotherapy in Primary invasive Extramammary Paget's vulvar disease because they are still controversial.
We searched the Cochrane Gynecological Cancer Group Trials Register, Cochrane Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE database up to September 2015. Radiotherapy was delivered as a treatment in various settings: i) Radical in 28 cases (range: 60–63 Gy), ii) Adjuvant in 25 cases (range: 39–60 Gy), iii) Salvage in recurrence of 3 patients (63 Gy) and iv) Neoadjuvant in one patient (43.3 Gy). A radiotherapy field that covered the gross tumor site with a 2–5 cm margin for the microscopic disease has been used. Radiotherapy of the inguinal, pelvic or para-aortic lymph node should be considered only for the cases with lymph node metastases within these areas.
Radiotherapy alone is an alternative therapeutic approach for patients with extensive inoperable disease or medical contraindications. Definitive radiotherapy can be used in elderly patients and/or with medical contraindications. Adjuvant radiotherapy may be considered in presence of risk factors associated with local recurrence as dermal invasion, lymph node metastasis, close or positive surgical margins, perineal, large tumor diameter, multifocal lesions, extensive disease, coexisting histology of adenocarcinoma or vulvar carcinoma, high Ki-67 expression, adnexal involvement and probably in overexpression of HER-2/neu. Salvage radiotherapy can be given in inoperable loco-regional recurrence and to those who refused additional surgery.
Extramammary Paget's disease (EMPD) is an intraepithelial carcinoma usually occurring on the skin or mucosa of the perineum. Clinically, it resembles eczema or dermatitis, and misdiagnosis and treatment delays are common. The treatment of choice for EMPD is a wide excision with adequate margins. Wide excision with intraoperative frozen biopsy and Mohs micrographic surgery are common methods; however, these are associated with a high recurrence rate and long operation time, respectively.
Between January 2010 and June 2013, 21 patients diagnosed with EMPD underwent mapping biopsy. Biopsy specimens were collected from at least 10 areas, 2 cm from the tumor margin. When the specimens were positive for malignancy, additional mapping biopsy was performed around the biopsy site of the positive result, and continued until no cancer cells were found. Based on the results, excision margins and reconstruction plans were established preoperatively.
The patients (18 male, 3 female) had a mean age of 66.5 years (range, 50-82 years). Almost all cases involved in the perineal area, except one case of axillary involvement. Permanent biopsy revealed one case (4.8%) of positive cancer cells on the resection margin, in which additional mapping biopsy and re-operation was performed. At the latest follow-up (mean, 27.4 months; range, 12-53 months), recurrence had not occurred.
Preoperative mapping biopsy enables accurate resection margins and a preoperative reconstructing plan. Additionally, it reduces the operation time and risk of recurrence. Accordingly, it represents an effective alternative to Mohs micrographic surgery and wide excision with intraoperative frozen biopsy.
Extramammary Paget’s Disease (EMPD) is a rare neoplastic lesion, which represents less than 1% of vulvar neoplasms. The lesion generally appears as eczema and the most frequently reported symptom is the itch. Also because of these poor clinical features, there is usually a delay in its diagnosis, based on the typical biopsy histological pattern. It has a good prognosis in absence of malignancy, but can result in a heavy quality of life impairment because of frequent recurrence with necessity of ablative therapies and anxiety for possible cancerization. Rarely EMPD can be invasive or associated to adenocarcinoma or other kinds of cancer. The first choice therapy is the surgical excision, with inguinal lymphadenectomy in case of infiltrative disease. However, many other conservative therapies, including the topical use of antiblastic, immuno-modulating, and hormone-modulating drugs, are used against EMPD even if still off label. After completion of this article, the reader should be able to recall the clinical manifestations of the EMPD, the histological pattern which allows its diagnosis, and to state the options for a treatment, which should be conservative and at the same time as radical as possible.
Apocrine carcinoma is a rare malignancy with invasive potential. It presents as painless, slow-growing, firm or cystic, red nodules with focal ulcerations. The tumor is capable of hematogenous dissemination to the liver, lungs, and bone as well as lymphatic spread. In addition, apocrine carcinomas cause intra-epidemial pagetoid spread. We report a case of an apocrine carcinoma related with extensive extramammary Paget's disease (EMPD). The relationship between apocrine carcinoma and EMPD remains to be understood. Co-existing cases with apocrine carcinoma and EMPD are discussed to better understand the relationship between these two malignant apocrine tumors.
This cancer shares many histologic similarity with Paget's disease of the breast. Clinically, patients present with itchy pink to red scaly areas, resembling a chronic eczema. There may be scattered islands of white hyperkeratosis. These areas may be scattered and multicentric, involving the vulva and perineum. It is a slow growing and progressive cancer which requries a wide local excision. In addition, there are occasional cases which are really underlying invasive adenocarcinomas which have secondarily spread to the overlying skin in 10-20% of cases. Underlying adenocarcinoma in 10-20% of cases represent secondary metastasis to the skin, not true extramammary Paget's disease. In these cases, the pathologist must perform a diligent search to identify a primary adenocarcinoma.