Frequently Asked Questions (FAQ)
Discover clarity and reassurance in our Frequently Asked Questions (FAQ) section designed specifically for extramammary Paget's disease (EMPD) patients and their families. From understanding symptoms EMPD and diagnosis to exploring treatment options, our answers provide invaluable insights rooted in compassion, and written by patients who have experienced EMPD.
Q – Is there an Extramammary Paget’s Disease (EMPD) Support Group?
A – Yes. The myEMPD support group was started many years ago to provide a safe and welcoming environment for EMPD patients. The myEMPD support group is open to anyone who has been diagnosed with extramammary Paget’s disease (EMPD), as well as their loved ones, and care givers. For more information review the extramammary Paget’s disease (EMPD) Support Group page.
Q – What is Extramammary Paget’s disease (EMPD)?
A – Extramammary Paget's Disease (EMPD) is a rare cancer that typically affects regions abundant in apocrine sweat glands, such as the genital area, perianal region, and axilla (armpit). It arises from abnormal Paget cells within the skin. Interestingly, EMPD cells exhibit cellular similarities to those found in Paget's breast cancer, despite occurring in different parts of the body. Given its rarity and potential complexity, consult a healthcare professional if you notice EMPD symptoms or receive an EMPD diagnosis.
Q – Is Extramammary Paget’s Disease (EMPD) cancer?
A – Extramammary Paget's disease (EMPD) is a rare skin cancer, often described as intraepithelial adenocarcinoma due to its manifestation within the epithelial layer of the skin. Intraepithelial indicates the layer of cells that form the skin surface, while adenocarcinoma refers to cancer that originates in glands. EMPD typically arises near apocrine (sweat) glands, predominantly affecting regions such as the genitalia, perianal area, and axilla (armpit). However, in very rare cases, EMPD can occur in other parts of the body. Early recognition of EMPD is crucial as its diagnosis is often delayed, potentially allowing for invasive progression, into the dermis or lymph nodes, if left untreated. Invasive EMPD may also signify underlying malignancies, underscoring the importance of ongoing follow-up with knowledgeable medical professionals to ensure timely intervention and optimal management strategies.
Q – What is the survival rate for those diagnosed with EMPD?
A – The prognosis for patients diagnosed with primary extramammary Paget's disease (EMPD) is generally good, with factors such as early diagnosis, treatment received, patient age, and the extent and depth of the EMPD playing crucial roles. Most patients receiving treatment can expect to lead normal or near-normal lives, especially when EMPD is confined to the epidermal layer and surgically removed with clear margins.
However, it's important to note that around 10 to 30% of primary EMPD cases may develop secondary cancers, either before, simultaneously, or after EMPD diagnosis. These secondary cancers can range from other skin malignancies to internal tumors, significantly impacting mortality rates, especially when there's underlying cancer, perianal involvement, invasion into the dermis, or lymph node spread.
The location of EMPD often correlates with the associated secondary cancer; for instance, perianal EMPD may relate to lower gastrointestinal cancers, while genital EMPD could signal urinary tract malignancies. Additionally, secondary cancers associated with EMPD may include breast, ovarian, bile duct, liver, renal cell, lung, stomach, pancreatic cancers, or regional lymph node involvement. EMPD can also spread contiguously from the original site, particularly from genital, urinary tract, or anorectal areas.
Q – How is EMPD diagnosed, and what tests will I need?
A – Diagnosing Extramammary Paget's Disease (EMPD) typically involves a comprehensive approach that includes clinical examination, biopsy, and potentially imaging studies. During the clinical assessment, your healthcare provider will carefully examine the affected area, looking for characteristic signs such as redness, itching, scaling, or ulceration. A biopsy is then essential to definitively confirm the diagnosis of EMPD. This procedure involves taking a small tissue sample from the affected area, which is sent to a laboratory for microscopic examination by a pathologist. Detection of Paget cells within the skin tissue confirms the presence of EMPD.
Depending on individual circumstances and the extent of the disease, additional imaging studies such as ultrasound, MRI (magnetic resonance imaging), or CT (computed tomography) scans may be recommended. These imaging tests can help assess the spread of the disease, particularly if there are concerns about involvement of deeper tissues or nearby lymph nodes. The specific diagnostic tests needed for EMPD can vary based on factors such as lesion location and severity. Your healthcare provider will tailor the diagnostic approach to your specific needs to ensure an accurate diagnosis and appropriate treatment plan.
Q - Do you know of any standard treatments for EMPD?
A - The rarity of extramammary Paget's disease (EMPD) often leaves physicians dependent on research studies for treatment guidance, as firsthand encounters are infrequent. This lack of direct experience contributes to significant variation in treatment approaches, compounded by institutional preferences influenced by medical staff expertise. Additionally, the location and size of the EMPD lesions play a crucial role in determining the most suitable treatment approach, leading to further variation in treatment modalities.
In seeking medical assistance for EMPD, men often turn to dermatologists or urologists, though this may vary depending on geographical regions. Conversely, women commonly seek guidance from gynecologic oncologists and dermatologists. In cases where EMPD affects areas near the anus, consultation with a colorectal surgeon or colorectal oncologist may be necessary. Other specialists, such as oncologists, plastic surgeons, or reconstructive urologist surgeons, may also be involved in the comprehensive care and treatment of EMPD.
In North America, surgical interventions, including Mohs micrographic surgery or wide local excision, or a combination of both, are often favored for treating EMPD. However, alternative treatment modalities may be considered, including topicals, especially when surgical options are limited. It's noteworthy that treatment approaches for EMPD can vary significantly between countries. Early detection remains pivotal for achieving favorable treatment outcomes, underscoring the importance of regular medical monitoring.
Given the wide variability in EMPD care, consultation with multiple healthcare professionals in a multidisciplinary team setting has proven beneficial for some EMPD patients. Seeking guidance from physicians experienced in treating EMPD can also enhance treatment outcomes. Recurrence is a common occurrence in EMPD cases, emphasizing the necessity for vigilant monitoring and prompt identification of any changes. Additionally, individuals with EMPD should remain vigilant for signs of other potential cancers, highlighting the importance of comprehensive medical management and ongoing surveillance.
Q – How do I know IF i have extramammary Paget’s disease (EMPD)?
A – Extramammary Paget's disease (EMPD) often starts as a rash like eczema around the genital, anal or perineum areas of women and men. (EMPD can also appear in the axilla (armpit) or in the rarest of cases other areas as well.) The most common symptoms include red, scaly or crusty areas around the genital or anal area. These locations often itch. Some with EMPD describe symptoms as feeling like they have "Jock Itch".
The easiest way to determine if you have extramammary Paget’s disease (EMPD) is to have dermatologist, urologist, gynecologist or surgeon examine you. In some countries, other types of physicians may also be familiar with EMPD. If they suspect EMPD they may take a punch or shave biopsy. Pathologists use special immunohistochemical stains to be able distinguish EMPD biopsies. Read More →
Q – Is extramammary Paget’s disease (EMPD) contagious?
A – Extramammary Paget’s disease (EMPD), a cancer arising from mutated cells, isn't contagious on its own, as it's not caused by infectious agents. However, if someone has EMPD alongside other illnesses, they may need to consider potential infections. Though EMPD itself isn't contagious, individuals with cancer should consult healthcare professionals for tailored guidance on managing their health.
Q – What follow-up treatments ARE RECOMMENDED AFTER being diagnosed with EMPD?
A – Treatments and follow-up for extramammary Paget’s disease (EMPD) differ and there is currently no standards of care. Often follow-ups include: regular visits with physicians that are knowledgeable about EMPD, full skin examinations, palpation of lymph nodes, ultrasound of lymph nodes, computed tomography (CT) scan or positron emission tomography (PET) scan, sigmoidoscopy or colonoscopy, endoscopy, cystoscopy, and blood tests such as a carcinoembryonic antigen (CEA) screening. Female EMPD patients may want to consider a Papanicolaou smear and mammography. Male EMPD patients, may want to consider a PSA blood test. After the first year or two of non-reoccurrence, visits and tests are sometimes reduced in frequency. Since EMPD cases vary, it is important to work with your physician to determine the best follow-up plan for your situation. This article, Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease, authored by over 50 doctors, is the most comprehensive medical paper to date discussing EMPD treatment.
Q – What is the prognosis for EMPD, and what are the chances of recurrence?
A – The prognosis for EMPD can vary depending on factors such as the stage of the disease, the effectiveness of treatment, and individual factors such as age and overall health. Recurrence rates vary but tend to be higher in cases where the disease was extensive or involved deeper tissue layers. Regular follow-up appointments with your healthcare team are essential for monitoring for any signs of recurrence.
Q – How Common is EMPD?
A – Extramammary Paget's disease (EMPD) is exceptionally rare, with its occurrence influenced by factors such as geographical location, race, and biological disparities. It predominantly afflicts older adults, typically diagnosed around the age of 66. Studies suggest that EMPD is most prevalent in Asians (10 cases per million), followed by Europeans (7 cases per million), and Westerners (0.9 cases per million). Across most regions, women are more prone to EMPD, except in Asia where men seem to be affected more frequently than women. Nevertheless, EMPD may be underreported due to its characteristic presentation in anatomically sensitive areas like the genitalia, perianal region, and axilla, alongside the advanced age at which it commonly manifests.
Q – Will EMPD affect my daily life and activities, such as work or exercise?
A – The impact of EMPD on daily life can vary depending on the extent of the disease and the treatments received. Some individuals may experience limitations in activities such as exercise or work, especially during treatment and recovery periods. It's essential to communicate with your healthcare team about any concerns or challenges you may face, as they can provide guidance and support.
Q – Is EMPD hereditary, and are there any genetic factors I should be aware of?
A – Extramammary Paget's disease (EMPD) is generally not hereditary, although genetic factors may contribute to its development as there are cases of EMPD within families. While specific genetic markers have not been identified, ongoing research aims to uncover potential genetic associations. If you have a family history of EMPD or are concerned about genetic predisposition, consulting with a healthcare professional or genetic counselor can provide personalized guidance. Despite the absence of clear genetic links, regular medical check-ups are essential for early detection and effective management of EMPD, ensuring prompt intervention if symptoms arise.
Q – Can I donate to an EMPD or cancer cause?
A – This website is personally funded by EMPD patients and is not registered as a charitable institution. As such we are not able to accept donations. However, here are several cancer-related organizations that do take donations and who would love to receive your charitable gifts. They include:
Friend for Life Cancer Support Network
4003 Kresge Way, Suite 100
Louisville, KY 40207-4652
Phone: 502-893-0643
Questions: staff@friend4life.orgFred Hutchinson Cancer Research Center
1100 Fairview Ave. N., Mail Stop J5-200
Seattle, WA 98109
Phone: 800-279-1618
Questions: annualgiving@fredhutch.orgMemorial Sloan Kettering Cancer Center
1275 York Ave.
New York, NY 10065
Phone: 866-815-9501
Questions: development@mskcc.org
Q – Who was Paget?
A – Sir James Paget, an English surgeon and pathologist, is renowned for his pivotal contributions to medical pathology. His legacy encompasses a range of Paget diseases, marking him as a pioneer in the field. Notable among his discoveries are Paget's disease of the nipple, Paget's disease of the bones, extramammary Paget's disease, and Paget's abscess. Paget's illustrious career spanned from 1851 until his passing in 1899, during which he dedicated himself to the advancement of medicine.
Extramammary Paget's disease (EMPD) first came to light through Paget's pioneering work in 1874. Subsequently, in 1889, Dr. Radcliffe Crocker expanded on this knowledge, specifically identifying cases affecting the penis and scrotum. Later, in 1893, doctors Darier and Coulillaud furthered our understanding by documenting EMPD occurrences in the perianal region.
It's worth noting that Extramammary Paget's disease (EMPD) shares cellular similarities with Paget's disease of the nipple, distinguishing them from Paget's other discoveries, which are unrelated.
Q – How Do YOU deal with the anxiety OF being diagnosed with a rare cancer?
A – Coping with the physical discomfort and psychological challenges linked to extramammary Paget’s disease (EMPD) and its treatments can be highly individualized. Given the nature of EMPD, it's not uncommon for patients to experience anxiety, depression, or post-traumatic stress disorder (PTSD) following diagnosis and treatment. If you find yourself grappling with these outcomes, it's crucial to open up about your experiences with trusted individuals such as family members, healthcare providers, clergy, or others who can provide support and guidance. Some EMPD patients have found that prayer, meditation, and exercise have also been helpful in dealing with EMPD. In addition, sharing your struggles can help alleviate feelings of isolation and empower you to navigate the emotional complexities of living with EMPD.
Q – Is There a Seach Feature on This Website?
A – Yes. A myEMPD.com search box is located below. There is also a search that is limited to just EMPD studies available here.
The content presented on this website, encompassing both textual material and visual media, serves educational and informational purposes aimed at aiding individuals affected by extramammary Paget’s disease (EMPD) and their families in comprehending the complexities associated with EMPD. It is imperative to note that the website is not designed to serve as a replacement for professional medical advice, diagnosis, or treatment. Furthermore, the website does not advocate for or endorse any specific treatments, tests, physicians, procedures, or products. Should you suspect that you are experiencing symptoms of EMPD, we strongly advise you to seek guidance from your healthcare provider.
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