Characterized by excessive breakdown of bonetissue followed by abnormal bone formation. Histological diagnostic confusion can arise between paget s disease and other neoplastic conditions affecting the skin, with the most common differential. Extramammary paget disease, abbreviated empd, is a skin disease. Metastatic extramammary pagets disease of scrotum responds. 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. Primary extramammary invasive pagets vulvar disease. 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. Common sites of occurrence include the vulva, perianal region, perineum, and scrotum. Wide surgical excision represents the standard treatment approach for primary invasive extramammary pagets vulvar disease. Mammary and extramammary paget s disease are uncommon intraepithelial adenocarcinomas. 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 paget disease definition of extramammary paget. Extramammary paget s disease epd is a rare variant of classical paget s disease, which accounts for about 6 % of all cases of paget s disease and about 1.
As the name suggests, there is also a paget disease of the breast there is also a paget disease of the bone just to make things confusing. Differential diagnosis in dermatopathology ii modern pathology. In addition to the scrotum, extramammary pagets disease empd can also arise in the groin vulva or penis and perineum region. Due to underlying carcinoma in 10 20% of cases of vulvar disease. A 62yearold man presented with a slowly progressive, asymptomatic lightbrown plaque on his back. 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. Extramammary paget disease actas dermosifiliograficas. Although empd is usually noninvasive and treated with local therapy, once metastatic the prognosis of empd is poor and treatment options are limited. Mar 02, 2018 secondary cutaneous extramammary pagets disease is termed so, when it develops secondary to systemic disease.
The full text of this article is available as a pdf 121k. Advanced extramammary pagets disease of the groin, penis. Before treatment begins, associated malignancies should be investigated. Background and objectiveextramammary paget disease empd has seldom been studied in mediterranean populations. Extramammary paget disease of the skin is a rare intraepithelial cancer characterised by a chronic eczemalike rash around the axillae or anogenital regions of males and females. Fortyeight patients were treated by surgical resection. Perianal pagets disease ppd involving perianal skin or anal mucosa accounts for less than 20% of extramammary pagets disease. The goal of the current study was to analyze the appropriate indications of radiotherapy in primary invasive extramammary pagets vulvar disease because they are still. Extramammary pagets disease rare cancers other conditions are entertained before the.
The lesion may be accompanied by an invasive adenocarcinoma or adenocarcinoma in situ of the apocrine glands. Labia majora, scrotum and perineum are most common sites. Extramammary paget s disease empd is a rare cutaneous neoplasm that is thought to represent intraepithelial adenocarcinoma developing in an area rich in apocrine glands. We report a case of a complete response to single agent trastuzumab in a hemodialysis patient with metastatic her2neu overexpressed empd of the scrotum. Extramammary paget disease of the scrotum with features of. This cancer is slowgrowingsometimes it is present for 10 to 15 years before it becomes noticeable. Extramammary pagets disease empd, is a rare and slowgrowing malignancy which occurs within the epithelium and accounts for 6. Extramammary pagets disease is more common in women than in men, and the vulva is the most common location. Mammary paget disease pd of the nipple was first described by sir james paget in 1874. Ayala, in urologic surgical pathology fourth edition, 2020. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. 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. May originate from intraepidermal portion of sweat glands or primitive basal cells with ability to differentiation towards glandular elements. Response to 5fluorouracil in metastatic extramammary paget disease of the scrotum presenting as pancytopenia and back pain.
The more problematic situation is differentiating primary anogenital extramammary paget s disease from secondary intraepidermal involvement from an underlying visceral malignancy. Since empd is so rare, it is often difficult to find a physician that is. Printable extramammary paget disease surgical pathology. This disambiguation page lists articles associated with the same title. Extramammary paget disease refers to nonbone, nonbreast paget disease. If an internal link led you here, you may wish to change the link to point directly to the intended article.
This fact sheet deals with paget s disease of the nipple, where there is no sign of any other cancer in the breast apart from in the nipple and surrounding area areola. Histopathologically, the characteristic cells of extramammary pagets disease show ample and pale cytoplasm and round and pleomorphic nuclei, which often shows prominent nucleoli. We describe the case of a man in his 50s who initially presented with a small eczematous lesion on his right groin, treated with topical ointments for eczema, until. Introductionpagets disease is a chronic condition of bonecharacterized by disorder of the normal boneremodeling process. 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 11, 2014 extramammary pagets disease empd is a rare intraepithelial malignancy with poorly described outcomes. 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. Oct 02, 20 paget s disease, described by sir james paget in 1874, is classified as mammary and extramammary. This is dealt with in the bone article and has nothing from a pathologic perspective to do with the paget disease discussed in this article. Extramammary paget disease empd is an uncommon condition that usually presents in apocrine sweat glandrich areas, most. Of greater rarity, however, is the occurrence of this pathological entity.
Extramammary pagets disease an overview sciencedirect topics. Extramammary pagets disease empd extramammary pagets disease empd is a rare intraepithelial adenocarcinoma skin cancer that can appear around the genitals, anus, perineum, groin or arm pit areas in women and men. Extramammary pagets disease is an uncommon intraepidermal malignant neoplasm that arises in area rich in apocrine glands. Dec 14, 2018 pagets disease of the scrotum is an extremely rare form of pagets disease, with an extramammary origin. Apr 18, 2017 a proposal for a tnm staging system for extramammary paget disease. The mammary type is rare and often associated with intraductal cancer 93100% of cases. Extramammary pagets disease that occurs in nonapocrinebearing regions is referred to as ectopic and has been rarely reported. Primary cutaneous forms exist associated with underlying adnexal carcinoma, in addition to forms associated with underlying anal, rectal or. Extramammary paget s disease epd is a rare variant of classical paget s disease, which accounts for about 6 % of all cases of paget s disease and about 1 % of neoplasms with vulvar location 105. Usually diagnosed on skin punch or shave biopsy of the nipple. Extramammary paget disease empd is a locally aggressive cutaneous malignancy that usually arises in anogenital or axillary skin. Extramammary paget disease surgical pathology criteria.
In a majority, the sites involved are in the abdominalpelvic region the urinary bladder, large intestine, and rectum. Extramammary pagets disease of the scrotum sciencedirect. 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. 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. Under the microscope, this condition looks very similar to a condition that occurs on the breast called mammary paget disease. Extramammary paget disease genetic and rare diseases. Retrospective analysis of 301 patients with invasive primary tumors. Paget s disease of the breast is a type of cancer that outwardly may have the appearance of eczema, with skin changes involving the nipple of the breast. Under the microscope, extramammary paget disease looks very similar to the more common mammary paget disease that occurs on the breast. In the rarest of rare cases it can appear elsewhere in the body. Jul 29, 2016 primary invasive extramammary pagets vulvar disease is a rare tumor that is challenging to control. Paget s disease of the nipple comprises approximately 2% of all breast cancers in women.
Mammary and extramammary pagets disease journal of. Expression of pd1 and pdl1 in extramammary paget disease. The similarities between extramammary paget disease empd and bowen disease have been described in the literature since bowen published his original article in 1912. Mar 24, 2020 extramammary pagets disease is a rare neoplastic condition of apocrine glandbearing regions 1, 2. Perianal paget s disease may also develop as an extension of perineal paget s disease in postmenopausal white women. Treatment of primary vulvar paget disease with 5% imiquimod cream. Extramammary paget disease empd can be defined as an intraepithelial. Intraepithelial involvement of the skin by nonsquamous carcinoma cells. 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. The clinical presentation of this disease is similar to the characteristics of mammary pagets disease mpd. The most frequently affected site is vulva, followed by perineal, perianal, scrotal and penile skin. However, unlike mpd, which occurs in large lactiferous ducts and then. Empd in men is primarily found in the perianal and penoscrotal areas.
881 1343 290 221 721 533 925 718 31 1511 448 874 576 1609 793 137 1417 638 460 953 1439 51 515 354 312 1159 1609 34 1051 520 589 128 552 78 596 1265 199 1475 1138 773 185 28 868 1151 1096 118 687 1255 658