It was first suggested by weiner in 1937 to be a carcinoma of the apocrine sweat gland with intraepithelial excision. Extramammary pagets disease empd is a rare cutaneous neoplasm that is thought to represent intraepithelial adenocarcinoma developing in an area rich in apocrine glands. Paget s disease of the nipple a form of intraductal breast cancer spreading into the skin around the nipple, and extramammary paget s disease. In exceptionally rare cases, empd can appear elsewhere in the body.
Unlike mammary paget disease, most cases of extramammary paget disease do not arise in association with an underlying malignancy, but occasional cases are associated with underlying carcinoma either adnexal or visceral, including cancers of the urinary bladder, prostate, rectum, urethra, and sweat glands. The most frequently affected site is vulva, followed by perineal, perianal, scrotal and penile skin. In the rare instances, extramammary pagets disease is a secondary event caused by extension of an adenocarcinoma. Lesions clinically present as erythematous, welldemarcated plaques that may become ulcerated, erosive, scaly, or eczematous. Vulva is by far the commonest anogenital site with perianal involvement being a distinct rarity. Extramammary pagets disease empd is a rare form of intraepithelial skin adenocarcinoma affecting most commonly the vulva. This is dealt with in the bone article and has nothing from a pathologic perspective. Immune checkpoint inhibitors targeting programmed cell death receptor pd1 andor its ligand pdl1 are approved for the treatment of several types of cancer, and response to these generally correlates with increased pdl1 expression by tumor cells. Extramammary pagets disease is more common in women than in men, and the vulva is the most common location. High risk of proximal and local neoplasms in 2206 patients. Most commonly extramammary pagets disease empd symptoms appear in genital, perineum, anal or armpits areas of women or men. Extramammary pagets disease empd is a rare skin cancer that can appear around the genitals, anus, perineum, groin or arm pit areas in women and men.
Even though both mpd and empd may appear similar clinically and histologically, they differ in their pathogenesis and frequency of associations with underlying malignancies. Extramammary paget disease can be separated into primary and secondary types primary type not associated with underlying deep carcinoma positive for gcdfp15 and negative for ck20 see supplemental studies and differential diagnosis at left sidebar. Nov 14, 20 extramammary paget s disease empd is a rare condition of the skin that often involves the vulva, perianal region, scrotum, penis, and axilla. A primer on extramammary pagets disease for the urologist. Apr 18, 2017 at clinical examination, extramammary paget disease empd may appear as chronic intertrigo or presumed tinea cruris. If an internal link led you here, you may wish to change the link to point directly to the intended article. Due to underlying carcinoma in 10 20% of cases of vulvar disease. Extramammary paget disease is characterized by the consistent. The primary difference between the two is the location of the rash. Extramammary pagets disease versus lichen sclerosus in. The more problematic situation is differentiating primary anogenital extramammary paget s disease. An immunoperoxidase study of gross cystic disease fluid protein15, carcinoembryonic antigen, and keratin proteins. Paget s disease of the nipple comprises approximately 2% of all breast cancers in women.
Palefsky, in diagnostic gynecologic and obstetric pathology third edition, 2018. May originate from intraepidermal portion of sweat glands or primitive basal cells with ability to differentiation towards glandular elements. Because of the lowfrequency of the disease, no specific guidelines about the treatment strategy are available. Mammary and extramammary paget disease of the skin images. Of greater rarity, however, is the occurrence of this pathological entity in areas other than the breast. Given the rarity of the disease there is a paucity of data to optimize treatment.
Labia majora, scrotum and perineum are most common sites. Breast tumours and tumours originating from apocrine glands stain with gcdfp15. Mar 08, 2016 extramammary paget disease empd is characterized by a chronic eczemalike rash of the skin around the genital regions of males and females. It may be multifocal and usually extends beyond clinically apparent margins. The clinical presentation of this disease is similar to the characteristics of mammary pagets disease mpd. Histological diagnostic confusion can arise between paget s disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous. Extramammary paget disease, abbreviated empd, is a skin disease. Extramammary paget s disease empd is a rare skin disease with a tendency to recur even in the face of adequate excision.
Extramammary paget disease genetic and rare diseases. Extramammary paget disease empd is characterized by a chronic eczemalike rash of the skin around the genital regions of males and females. Radiation oncologyempd wikibooks, open books for an. Extramammary paget s disease empd is a rare yet lethal cutaneous malignancy with an overall survival rate of 60.
Primary cutaneous forms exist associated with underlying adnexal carcinoma, in addition to forms associated with underlying anal, rectal or. Jul 06, 2014 extramammary pagets disease empd is a rare intraepithelial malignancy arising in areas rich in apocrine glands, such as the perineum, vulva, axilla, scrotum, and penis. Paget disease pathological process occurs in four stages. With your help, we can update and expand the website. Extramammary paget s disease is a rare neoplastic condition of apocrine glandbearing regions 1, 2. Anatomic pathology, 12605 e 16th ave, campus box f768, aurora, co 80045. Melanoma in situ in contrast to paget, melanoma is negative for cytokeratins, ema. Under the microscope, extramammary paget disease looks very similar to the more common mammary paget disease that occurs on the breast. Rare cases of basal cell carcinoma, sebaceous carcinoma, apocrine carcinoma, adenoid cystic carcinoma, and spiradenocarcinoma were identified.
The purpose of this report is to describe an unusual case of extramammary paget s disease with urethral and lymph node infiltration and demonstrate the role of mri in the preoperative period for. Thus, extramammary pagets disease involves areas in which apocrine glands are normally encountered. Histological diagnostic confusion can arise between paget s disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant. Advanced extramammary pagets disease of the groin, penis. In the rarest of rare cases it can appear elsewhere in the body. To investigate a possible follicular origin of extramammary paget s disease epd. However, unlike mpd, which occurs in large lactiferous ducts and then extends into the epidermis, empd originates in glandular regions rich in. Paget disease occurs when theres an increase of bone resorption that leads to a decrease in bone mass and lytic structures.
Paget disease, vulva, neoangiogenesis, emt, microvessel density, hvd, avd background extra mammary paget s disease of the vulva empdv is an infrequent chronic disease that often recurs and accounts for less than 1% of vulvar neoplasms 1. Extramammary paget disease is a malignant tumor typically found in. We present a case of a 59yearold woman with primary extramammary paget s disease of the vulva, in which a conservative approach to therapy was desired, and who underwent complete and stable. The current mainstay of treatment is wide local excision. This disambiguation page lists articles associated with the same title. 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. Ls and extramammary paget s disease empd belong to a group of uncommon dermatoses which mainly affect the skin of the genitals in postmenopausal women. The similarities between extramammary paget disease empd and bowen disease have been described in the literature since bowen published his original article in 1912. Women in their sixth to eighth decades are commonly affected. Since empd is so rare, it is often difficult to find a physician that is. Extramammary pagets disease an overview sciencedirect topics. In the largest published cohort of patients with extramammary paget s disease, patients with anal paget s had a much higher risk of both proximal and local neoplasms as compared with patients with genital paget s. Although skin wrinkles on mri may be misinterpreted based on observers experience, it correlates well with pathology and may provide an.
Empd mostly progresses slowly and is often diagnosed as carcinoma in situ. Paget disease may be classified as primary, thought to arise from apocrine glands, or secondary, resulting. Perianal paget s disease ppd involving perianal skin or anal mucosa accounts for less than 20% of extramammary paget s disease. We assessed incidence and time trends of empd in men as well as extent and predictors of survival using the surveillance, epidemiology, and end results seer program. Extramammary paget s disease empd is a rare, slowgrowing, cutaneous adenocarcinoma that usually originates in the anogenital area and axillae outside the mammary glands. His famous works included lectures on tumours 1851 and lectures on surgical pathology 1853. Although prognosis is generally favorable, it can be associated with neoplasms of the bladder, urethra, prostate, and rectum. S100, hmb45 and mart1 are usually negative in pagets disease and positive in melanoma. The lesion may be accompanied by an invasive adenocarcinoma or adenocarcinoma in situ of the apocrine glands. Formalinfixed, paraffinembedded tissue sections from 45 patients with mammary and extramammary paget s disease were stained immunohistochemically with the use of a polyclonal antiserum directed against a 14amino acid segment of the cerbb2 oncoprotein.
Nov 11, 2014 extramammary pagets disease empd is a rare intraepithelial malignancy with poorly described outcomes. Extramammary paget disease empd is a rare intraepithelial adenocarcinoma. May have cleftlike spaces between paget cells and neighboring keratinocytes. Extramammary paget disease of the skin of the axilla. Extramammary paget disease definition of extramammary paget. Objectives extramammary paget disease empd of the vulva is a rare lesion with a high recurrence rate ranging from 12% to 61%. The rate of underlying adenocarcinoma varies, but in the largest series was reported at 4%. Extramammary pagets disease empd, is a rare and slowgrowing malignancy which occurs within the epithelium and accounts for 6. Mammary and extramammary pagets disease journal of. Two types primary extramammary paget disease thought to be an intraepidermal carcinoma arising from cutaneous apocrine glands arises in apocrine rich areas usually the vulva but also the groin, inguinal area, perineum, penis or scrotum and rarely axilla or eye. Extramammary paget disease of the skin is a rare intraepithelial cancer characterised by a chronic eczema like rash around the axillae or anogenital regions of males and females. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. While most people recall paget s disease refers to bone, two other diseases were also named after him. Oct 02, 20 paget s disease, described by sir james paget in 1874, is classified as mammary and extramammary.
Surgery is the recommended therapy for resectable and localized disease, but several other local treatments have been reported such as radiotherapy. Extramammary pagets disease is an uncommon intraepidermal malignant neoplasm that arises in area rich in apocrine glands. Expression of pd1 and pdl1 in extramammary paget disease. Extramammary paget disease is a rare cancer related to paget s disease of the breast, but found around the anus and genitals of both men and women.
Further studies are needed to determine the real ef. It may appear eczematous, and it has usually been present for several years before biopsy is performed to confirm the diagnosis. It is characterized by peculiar cells socalled paget. Extramammary paget s disease is a rare cutaneous malignancy characterized by scaly red patches that are sometimes pruritic in apocrinebearing areas such as the axillae and groin. Mammary and extramammary paget s disease are uncommon intraepithelial adenocarcinomas. Extramammary paget disease of the skin is a malignancyassociated skin disease characterised by an eczematous rash of the anogenital region and vulva, where there is an abundance of apocrine glands. A proposal for a tnm staging system for extramammary paget disease.
Extramammary pagets disease an overview sciencedirect. Morphologically the same as paget s disease of the breast, but unlike in the nipple, there is only rarely an underlying malignancy. Extramammary pagets disease is a rare cutaneous neoplasm that occurs mainly in the elderly. Extramammary pagets disease bjrcase reports vol 2, no 2. Extramammary paget s disease, is a rare and slowgrowing malignancy which occurs within the epithelium and accounts for 6. By continuing to use our website, you are agreeing to our use of cookies. Extramammary paget disease surgical pathology criteria. S100 may be expressed by paget cells so it is not a perfect marker to rule out melanoma melana is better. The role of radiotherapy in extramammary paget disease. Extramammary pagets disease empd is a rare skin disorder involving adult caucasians. We present the case of a 56yearold woman with perianal empd that recurred 4 years after initial treatment with wide local excision with mohs micrographic surgery tissue processing of marginal tissue. Melanoma in situ in contrast to paget, melanoma is negative for cytokeratins, ema and cea. Perianal extramammary paget disease treated with topical.
Retrospective analysis of 301 patients with invasive primary tumors. Extramammary paget disease refers to nonbone, nonbreast paget disease. Objectivepurpose extramammary paget disease empd is a rare neoplasm of the skin generally affecting the anogenital area. Extramammary paget disease of the scrotum with features. To examine the results of treatment of extramammary pagets disease empd in ethnic chinese. The clinical presentation of this disease is similar to the characteristics of mammary paget s disease. Extramammary paget disease empd is a locally aggressive cutaneous malignancy that usually arises in anogenital or axillary skin. It presents by an eczematous rash of the anogenital region and vulva, areas of abundant apocrine glands.
Extramammary paget disease epd is an uncommon cutaneous malignant neoplasm that arises in areas rich in apocrine glands perineum, vulva, and axilla. Extramammary paget s disease empd is a clinically distinct condition that affects extramammary sites, such as the vulva, penis, scrotum, perineum, and anus. Paget skin disease an overview sciencedirect topics. Ectopic extramammary pagets disease affecting the lower anterior aspect of the chest. Extramammary pagets diseaseevidence for an apocrine origin.
Extramammary pagets disease of the scrotum sciencedirect. The mammary type is rare and often associated with intraductal cancer 93100% of cases. Treatment of primary vulvar paget disease with 5% imiquimod cream. Dermal invasion is rare in vulva, more common in perianal region single, clusters or glandular formations of large cells with pale, vacuolated cytoplasm, usually just above basal layer of epidermis. Unusual presentation of ectopic extramammary paget disease. Common sites of occurrence include the vulva, perianal region, perineum, and scrotum. Printable extramammary paget disease surgical pathology. Markers useful for separating primary from secondary paget disease gcdfp15 brst2 and ck20 are useful for separating primary from secondary paget disease.
This cancer is slowgrowingsometimes it is present for 10 to 15 years before it becomes noticeable. Extramammary paget disease epd is an uncommon cutaneous malignant neoplasm that arises in areas rich in apocrine glands perineum, vulva, and axill we use cookies to enhance your experience on our website. Introduction extramammary paget s disease empd is a distinct form of. In addition to the scrotum, extramammary pagets disease empd can also arise in the groin vulva or penis and perineum region. Extramammary paget disease was the most common 49 of 56, and 29% 14 of 49 demonstrated an invasive component. Response to 5fluorouracil in metastatic extramammary paget disease of the scrotum presenting as pancytopenia and back pain. Its incidence varies between less than 1% and 2% of vulvar malignancies, 1 with a higher incidence in postmenopausal white females. Under the microscope, this condition looks very similar to a condition that occurs on the breast called mammary paget disease. Apr 18, 2017 a proposal for a tnm staging system for extramammary paget disease.
Epd is a predominantly intraepidermal tumour with extensive involvement of adnexal structures and high recurrence rates suggesting a follicular stem cell origin. Extramammary pagets diseasea proliferation of adnexal. Empd in men is primarily found in the perianal and penoscrotal areas. Extramammary paget disease lacks the her2neu expression seen in mammary paget disease. This process gives rise to osteoblasts from the bone utilizing a sensing system that allows them to increase its activity. We describe the case of a man in his 50s who initially presented with a. As the name suggests, there is also a paget disease of the breast. Hi neela, i dont have fa or pathoma and therefore, i dont know what they have in the books but here is my take on empd. Empd generally affects postmenopausal women and men over the age of 40, with most cases occurring in individuals over 60. Extramammary paget disease is characterized by the. Extramammary pagets disease empd is a rare intraepithelial malignancy with poorly described outcomes. Extramammary paget disease empd is an uncommon condition that usually presents in apocrine sweat glandrich areas, most commonly the vulva followed by the perianal region. Extramammary paget disease empd is a rare intraepithelial carcinoma and an uncommon variant of paget disease affecting areas of the apocrinerich skin of the perineum, vulva, and less commonly, axilla.
The disease usually involves skin areas rich in apocrine glands such as axilla and anogenital region. It is more prevalent in postmenopausal women and it appears as. Intraepithelial neoplasm with glandular differentiation. Dec 14, 2018 pagets disease of the scrotum is an extremely rare form of pagets disease, with an extramammary origin. There is also a paget disease of the bone just to make things confusing. Extramammary paget disease is a rare cancer related to pagets disease of the breast, but found around the anus and genitals of both men and womenmost commonly on the vulva of postmenopausal women. This disease is distinct from paget s disease of the breast and predominantly affects the apocrine glandbearing skin such as the perianal, genital and axillary regions of the body. Histopathologically, the characteristic cells of extramammary pagets disease show ample and pale cytoplasm and round and pleomorphic nuclei, which often shows prominent nucleoli. Apocrine gland origin or apocrine differentiation of cells of epd has been suggested. Patients with anal paget s also experienced worse survival as compared with those with purely genital paget s. From the department of pathology, university of colorado anschutz medical campus, denver. The purpose of this report is to describe an unusual case of extramammary pagets disease with urethral and lymph node infiltration and demonstrate the role of mri in the preoperative period for the assessment, management and prognosis of the disease.
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