Extramammary Paget's Disease Of Vulva Research Articles

Imiquimod therapy for extramammary Paget's disease of the vulva: A viable non-surgical alternative

Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma that can affect the vulval skin. Surgical excision is the gold-standard treatment, however, recurrence rates are high and extensive excisions can produce long-lasting cosmetic and functional defects. We describe one of the largest case series to-date (n = 6) on the use of topical 5% imiquimod cream as a novel treatment option and discuss our experiences. With the addition of our six cases to the literature, there are now 29 documented cases of vulval EMPD treated with 5% imiquimod cream. Of these, 50% of primary disease cases and 73% of recurrent primary disease cases have achieved clinical resolution with 5% imiquimod therapy alone. These findings suggest that imiquimod provides a viable alternative to surgical excision for vulval EMPD. However, we acknowledge that this is a simple retrospective analysis and that treatment scheduling and follow-up needs investigation in a trial setting.

Extramammary Paget disease successfully treated with topical imiquimod 5% and tazarotene

Extramammary Paget disease (EMPD) is an intraepithelial adenocarcinoma usually localized in areas rich in apocrine sweat glands. Surgery remains the treatment of choice for EMPD. However, several nonsurgical treatments have been also described. Around 40 cases of EMPD treated with imiquimod 5% have been published; of these, only six correspond to nonresponses. We describe a recurrent vulvar EMPD with failure to respond to topical imiquimod 5% in monotherapy but a favorable response to its association with tazarotene.

Methyl - aminolevulinic acid photodynamic therapy and topical tretinoin in a patient with vulvar extramammary Paget's disease

Extramammary Paget's disease is a rare neoplasm of apocrine gland-bearing areas of the skin. The most common site of presentation is the vulva. Surgery is the most frequently reported therapy so far; however, it is invasive and it is complicated by a high rate of recurrence. For this reason, several less-invasive treatments have been recently proposed, including photodynamic therapy. We describe in this article the case of an 84-year-old patient with a noninvasive vulvar extramammary Paget's disease successfully treated with methyl-aminolevulinic acid photodynamic therapy associated with topical tretinoin.

Successful treatment of three cases of primary extramammary Paget’s disease of the vulva with Imiquimod – proposal of a therapeutic schedule

Extramammary Paget's disease (EMPD) is an intraepidermal adenocarcinoma of apocrine gland-bearing skin. Although surgery remains the mainstay of treatment, loss of tissue function and high recurrence rates have been reported. Recently, topical Imiquimod has been shown as a safe and effective treatment option for extramammary Paget's disease. Three patients diagnosed of EMPD of the vulva were treated with a daily application of 5% Imiquimod cream for three weeks, followed by an every other day application for an additional three weeks. Complete clinical and histological remission of the disease was achieved in the three patients. Mild irritation and tenderness were observed as the only side effects. Based on our results, we consider that Imiquimod cream is a safe and effective therapeutic option for the treatment of vulvar EMPD. These promising results warrant further studies to determine the real efficacy and safety of Imiquimod 5% cream for the treatment of this uncommon disease.

HER‐2/neu expression in extramammary Paget disease: a clinicopathologic and immunohistochemistry study of 47 cases with and without underlying malignancy

Extramammary Paget disease (EMPD) is an infrequent skin cancer sometimes representing a secondary event caused by extension of an underlying carcinoma. Her-2/neu overexpression in breast cancer is correlated with a more aggressive behavior, but anti-Her-2/neu therapy improves survival in these patients. We investigated Her-2/neu expression by immunohistochemistry in cases of EMPD with and without underlying malignancy to try to correlate with tumor recurrence, progression and possible targeted therapy. Forty-seven cases were analyzed (6 from the scrotum, 7 perianal region, 1 axilla and 33 vulva). Two cases had invasive EMPD (one from vulva and one from scrotum). The overall Her-2/neu expression was 31.9%. Of the noninvasive EMPD of the vulva (32 cases), Her-2/neu was shown in 38%. The case of invasive vulvar EMPD was negative. All six scrotal EMPD lacked Her2/neu expression. Her-2/neu was expressed in two of seven perianal cases (33.3%). The EMPD on the axilla (one case) was negative. Eighteen cases had recurrence, and of these, 44.4% expressed Her-2/neu in the initial lesion. A high proportion of EMPD showed Her-2/neu expression (31.9%), indicating that these patients may benefit from targeted therapy. The proportion of positive cases was higher in lesions that had recurred at last follow up (44.4%), suggesting a more aggressive behavior.

Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome

Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15- phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy.

Pagetoid Lesions of the Vulva

Vulvar extramammary Paget disease (EMPD) is an intraepithelial carcinoma not frequently seen in conjunction with other primary tumors. Nevertheless, we are presenting a collision between malignant nodular melanoma and EMPD in the vulva of a 78-year-old gravida 6, para 6 woman with a medical history of chronic lymphocytic leukemia. The patient presented with vulvar irritation and a pigmented vulvar mass; biopsy confirmed the diagnosis of malignant melanoma. Peripheral to the main mass, the vulva grossly seemed erythematous, and the perineum seemed white and thickened. Multiple radial biopsies were taken to determine the extent of involvement by malignant melanoma, and these biopsies revealed that the "irritated" areas surrounding the melanoma were EMPD. Subsequently, a radical vulvectomy was performed, the margins of which were clear of both neoplasms. Five months later, bilateral groin adenopathy was biopsy proven to be metastatic melanoma and chronic lymphocytic leukemia, and the patient died 8 months after the radical vulvectomy.

Flow cytometric DNA analysis of extramammary Paget's disease of the vulva.

Vulvar extramammary Paget's disease (EMPD) is an uncommon disease entity that occurs predominantly in postmenopausal white women. The clinical behavior of this neoplasm is extremely variable, reflecting the various histological patterns that have been reported with this lesion. Flow cytometry has been used as a method of obtaining prognostic information about a number of gynecological neoplasms, yet to date there have been no flow cytometric studies performed on this unusual neoplasm. We performed flow cytometric analysis of 14 cases of paraffin-embedded vulvar EMPD in patients ranging from 45 to 86 years of age. We correlated histological features and clinical recurrence risk with DNA analysis. Although we were unable to show a statistically significant correlation between DNA ploidy or S-phase and time to recurrence, we did show a statistically significant correlation between DNA ploidy and histological features. Aneuploidy appears to be associated with in situ sweat gland adenocarcinoma, invasive carcinoma, and lymphatic invasion. These results suggest that Paget's cells with aneuploid DNA stem lines may be associated with the potential for aggressive biologic behavior.