We assessed the p16 expression by immunohistochemistry in 40 cases of primary vulvar empd, including 34. Differential diagnosis in dermatopathology ii modern. 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. Hilliard, conway huang and aleodor andea, pigmented extramammary paget s disease of the axilla mimicking melanoma. Study of neuprotein expression in mammary paget s disease with and without underlying breast carcinoma and in extramammary paget s disease. The clinical presentation of this disease is similar to the characteristics of mammary paget s disease mpd. Twenty patients were identified from the pathology files of 4 institutions between 2000 and 2018. Its incidence varies between less than 1% and 2% of vulvar malignancies, 1 with a higher incidence in postmenopausal white females. Once a diagnosis of empd is made, the patient must be riskstratified and. Extramammary paget disease of the scrotum with features of. Extramammary paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Produces mucus that drains into vaginal canal via the lower vestibule. Pathology of mammary and extramammary paget s disease.
Extramammary pagets disease an overview sciencedirect topics. However, unlike mpd, which occurs in large lactiferous ducts and then extends into the epidermis, empd. The more problematic situation is differentiating primary anogenital extramammary paget s disease from secondary intraepidermal involvement from an underlying visceral malignancy. Empd mostly progresses slowly and is often diagnosed as carcinoma in situ. The results obtained in this study, coupled with data on the frequency of the supernumerary breasts, lead to the speculation that extramammary paget s cells originate from ectopic mammary glands or from pluripotential germinative cells in the epidermis, capable of differentiating toward the mammary glands. It can appear in a number of areas, including the perianal skin, external auditory canal, conjunctiva, umbilicus, axilla. Extramammary paget s disease empd, is a rare and slowgrowing malignancy which occurs within the epithelium and accounts for 6. This is often manifested by diffuse pain throughout the musculoskeletal system. This section lists our premium articles on topics related to death. Extramammary paget s disease empd is a rare cutaneous malignancy. Pagets disease chapter 9 pathology of bone and joint. Bone marrow mononuclear cells from patients with paget s disease contain measles virus nucleocapsid messenger ribonucleic acid that has mutations in a specific region of the sequence. Extramammary paget s disease is an adenocarcinoma of the epidermis that may resemble eczema.
Gynecologic pathology 9780323359092 us elsevier health. Extramammary paget disease genetic and rare diseases. Due to underlying carcinoma in 10 20% of cases of vulvar disease. Nodal and distant organ metastatic disease is seen. 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 womenmost commonly on the vulva of postmenopausal women. Cytokeratins, cea, and mucin histochemistry in the. Extramammary paget disease empd is a locally aggressive cutaneous malignancy that usually arises in anogenital or axillary skin. In men the penis and scrotum are most frequently involved, and in women the vulva.
Retrospective analysis of 301 patients with invasive primary tumors. The popular template format makes it easy to use, and new information throughout brings you up to date with whats new in the field. Extramammary paget disease empd is characterized by a chronic eczemalike rash of the skin around the genital regions of males and females. Even though both mpd and empd may appear similar clinically and histologically, they differ in their pathogenesis and frequency of associations with underlying malignancies. Anal or perianal paget s disease may occur in association with a distal adenocarcinoma of the rectum, the latter of which may be diagnosed before the anal paget s disease, at the same time, or later. 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 can be defined as an intraepithelial adenocarcinoma that primarily involves the epidermis outside. Extramammary pagets disease an overview sciencedirect. Extramammary paget disease of the vulva has been defined by the world health organization as an intraepithelial neoplasm of cutaneous origin expressing eccrine or apocrine glandularlike features and characterized by distinctive large cells with prominent cytoplasm referred to as paget.
The more experience a doctor has performing mohs surgery specifically with empd cases the better prepared they are to anticipate and prevent complications. The disease typically affects older individuals aged 6080 years and is seen most frequently in postmenopausal caucasian women. The main purpose of this manual is to provide useful guidelines for the selection of pathology tests and to facilitate interpretation of results. P16 immunohistochemistry has been widely used in facilitating the diagnosis of human papillomavirus hpvrelated usual type vulvar intraepithelial neoplasm. However, ck7 immunoreactivity should be interpreted carefully in the context of morphologic findings since it. This disambiguation page lists articles associated with the same title. Extramammary paget disease empd can be classified into primary empd in sensu stricto and secondary variants, with the latter representing secondary involvement from an underlying carcinoma originating most commonly in the lower gastrointestinal or urinary tract. Vaginal, vulvar, cervical pathology flashcards quizlet. Extramammary paget disease empd is a rare intraepithelial adenocarcinoma.
Paget disease may be classified as primary, thought to arise from apocrine glands. Paget disease is a skeletal growth disorder in which abnormalities such as unusual bone growth can occur in several multifactoral ways. The current mainstay of treatment is wide local excision. 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. Adenocarcinoma of the mammarylike glands of the vulva. Now fully revised to include recent advances in the field, the second edition of gynecologic pathology, a volume in the foundations in diagnostic pathology series, is an essential foundation text for residents and pathologists. Extramammary paget disease refers to nonbone, nonbreast paget disease. Extramammary paget disease, abbreviated empd, is a skin disease. If an internal link led you here, you may wish to change the link to point directly to the intended article.
Lesions clinically present as erythematous, welldemarcated plaques that may become ulcerated, erosive, scaly, or eczematous. This cancer is slowgrowingsometimes it is present for 10 to 15 years before it becomes noticeable. These cells show differentiation along sweat gland lines. Intraepithelial involvement of the skin by nonsquamous carcinoma cells. Extramammary paget s disease patients often look for physicians who are boardcertified in dermatology and routinely perform mohs surgery. Mammary paget s disease also called paget s disease of the nipple is a condition that involves the nipple and areolar complex. Fifteen years later crocker described the first case of extramammary pagets disease affecting. These cells have ample pale cytoplasm and large pleomorphic. Paget skin disease an overview sciencedirect topics. Extramammary paget disease surgical pathology criteria.
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. Labia majora, scrotum and perineum are most common sites. 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. Ayala, in urologic surgical pathology fourth edition, 2020. Perianal extramammary paget disease treated with topical.
The primary difference between the two is the location of the rash. 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. Immunohistochemical profile of mammary paget disease pd. Paget s extramammary disease is associated with lifethreatening complications related to the disease and its treatment. Extramammary pagets disease empd is a clinically distinct condition that affects extramammary sites, such as the vulva, penis, scrotum, perineum, and anus. Under the microscope, extramammary paget disease looks very similar to the more common mammary paget disease that occurs on the breast. Extramammary pagets disease empd, is a rare and slowgrowing malignancy which occurs within the epithelium and accounts for 6.
Campbell 2004 extramammary paget disease of the scrotum with features of bowen disease. A proposal for a tnm staging system for extramammary paget disease. 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. Vulva is the most common site of extramammary paget s disease. To identify a sensitive marker for extramammary pagets disease and to identify histochemical and immunohistochemical features that suggest occult pelvic cancer in patients with extramammary pagets disease, we retrieved all cases between 1983 and 1992 with a standardized nomenclature of medicine code of extramammary pagets disease in the. Extramammary pagets diseasea proliferation of adnexal. Primary empd is considered an analogue of mammary paget disease mpd. Extramammary paget disease is characterized by the. Printable extramammary paget disease surgical pathology.
May originate from intraepidermal portion of sweat glands or primitive basal cells with ability to differentiation towards glandular elements. In empd, the proliferative neoplastic cell is the paget cell pc. Pathology of extramammary pagets disease dr sampurna roy md. However, studies of p16 expression in primary vulvar extramammary paget disease empd are limited. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Paget s disease is often associated with underlying carcinoma of the breast. Primary cutaneous forms exist associated with underlying adnexal carcinoma, in addition to forms associated with underlying anal, rectal or. Extramammary paget disease statpearls ncbi bookshelf. Extramammary paget disease epd first appeared in the literature in 1889. Response to 5fluorouracil in metastatic extramammary paget disease of the scrotum presenting as pancytopenia and back pain. Skin nonmelanocytic tumor extramammary paget disease. Thus, extramammary pagets disease involves areas in which apocrine glands are normally encountered.
Palefsky, in diagnostic gynecologic and obstetric pathology third edition, 2018. Extramammary paget disease empd is the rarer nonbreast form of paget disease of the nipple it is considered a form of adenocarcinoma of the apocrine glandular tissue. It is considered to be insitu adenocarcinoma arising most probably from the multipotential cells located along the basal layer of the epidermis. 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. 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. Sir james paget first described pagets disease of nipple in 1874. Under the microscope, this condition looks very similar to a condition that occurs on the breast called mammary paget disease. Extramammary paget disease radiology reference article.
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