Summary: Vulva Paget’s disease, also known as extramammary Paget’s disease (EMPD), is a rare type of skin cancer that affects the vulva, perianal region, and other apocrine gland-bearing areas. It appears as an eczema-like rash and may be attributed to several underlying cancers.
1. What is vulva Paget’s disease?
Vulva Paget’s disease is a type of skin cancer that is very rare, affecting approximately 1% of all vulvar malignancies. Most often, it presents itself as an eczema-like rash with itching in the vulva or the perianal region. This condition can be confused with other skin conditions such as psoriasis or allergic reactions but should not be overlooked for this reason. As this condition advances, there can be ulcers and large red or gray skin lesions, which can be a signal of invasive associated carcinomas.
The precise cause of vulva Paget’s disease is unknown, but it is often linked with some underlying cancers. This condition is caused by abnormal cells or malignant skin cells forming on the surface of the skin. Treatment is necessary because of the involvement of associated invasive tumors and the high likelihood of recurrence.
Vulva Paget’s disease can be classified into two types: primary and secondary. Primary vulva Paget’s disease afflicts only the vulva, while secondary vulva Paget’s disease is caused by cancer involving other parts of the body and subsequently affects the vulva through direct extension or spread via the lymph vessels.
2. Symptoms of vulva Paget’s disease
The primary symptoms of vulva Paget’s disease include itching, tingling, burning, and soreness in the vulva region. Eczema-like, red, raw, or scaly patches of skin appear in the vaginal and anal region, which require biopsy. The texture is typically crusted, thick, or rough. Women reporting these symptoms should promptly see a specialist in vulvar diseases to differentiate Paget’s disease from other similar symptoms.
When cancer progresses, there may be lumps, swelling, and ulcers of the vulva region. Other cancer-related symptoms may include abnormal bleeding and discharge, which can be accompanied by pressure or pain in the pelvic area. In most cases, only one area of skin or area covering mucosa is involved, but it is possible for the disease to spread.
The symptoms of vulva Paget’s disease are often mistaken for other types of skin conditions, and many patients often go months or even years before receiving accurate diagnosis and treatment. This delay in diagnosis can result in a more severe and widespread disease.
3. How is Vulva Paget’s disease diagnosed?
Vulva Paget’s disease is diagnosed through physical examination and biopsy. In the case of suspected vulva Paget’s disease, a woman will be referred to a specialist in vulvar cancer. Tests may include punch or incisional biopsies, sentinel lymph node mapping and sampling to evaluate the spread of cancer cells into the lymph system.
If other underlying cancers are discovered, tests such as mammography, ultrasound, or MRI are used to check to see how far the cancer has advanced and if there have been any distant metastases. This type of diagnostic workup requires an interdisciplinary approach and a team of oncologists and specialists as this kind of disease carries a high risk of recurrence and progression. It should also be noted that the definitive diagnosis of vulva Paget’s disease relies on demonstrating presence of admixed benign and malignant cells consistent with epithelial malignancy and expressed by Paget malignant cells.
If you have any suspicion of cancer, it is important to seek help as soon as possible. Early detection is a significant factor in determining the success of treatment and increasing the likelihood of survival.
4. What is the treatment for vulva Paget’s disease?
The treatment options for vulva Paget’s disease depend on many factors, including the extent of the disease and its severity. Surgery is the mainstay of treatment of vulva Paget’s disease. Wide local excision of the area affected area as well as any associated tumors is recommended, with or without lymphadenectomy if necessary. Radiation therapy may also be recommended for patients with larger or recurrent lesions. Chemotherapy, used most often for advanced cancers, may also be recommended in some cases.
Vulva Paget’s disease does not respond well to conventional treatments such as antifungal or steroid creams. Instead, revolutionary treatments such as immunotherapy are being researched and constantly developed in medicine. Immunotherapy is a type of treatment that activates your body’s immune system to fight tumor cells more effectively, ultimately enhancing your natural defenses against disease.
It is common for women who have been diagnosed with vulva Paget’s disease to feel frightened, depressed, and anxious about their diagnosis and prognosis. It is essential to stay positive and take care of yourself mentally and physically. There are resources available such as support groups where you can connect with others undergoing similar experiences, either through your specialist or online.
5. What is the outlook for vulva Paget’s disease?
Vulva Paget’s disease is a slow-progressing cancer that can impact early detection, subsequently reducing the chances for curative treatment. Furthermore, it has a high incidence of local recurrence seen after surgical intervention. Depending on the size and stage of the tumor, the 5-year survival rate for vulva Paget’s disease can be up to 80%. Progress in treatment has led to advances in disease prognosis, with newer treatments improving the survival rates for women with the disease. However, long term follow-up is needed as late recurrences have been reported.
The severity of this disease is determined by many factors such as the location and size of the lesions, age and overall health of the patient, the extent of cancer cells activity, and the presence of other underlying conditions. It is vital to work with a specialist in vulvar cancer who can help tailor treatment options best suited for your needs.
Vulva Paget’s disease is a rare, slow-moving type of skin cancer that is often misdiagnosed. It affects only a small percentage of women but requires prompt attention when suspected. Timely diagnosis and treatment are essential to ensuring positive outcomes, while delays in treatment significantly increase the risks associated with this disease. Working with a trusted specialist in vulvar cancer can provide the insight necessary to manage this illness successfully. Vigilance and understanding are essential keys to defeating this disease, and women who stay positive about their recovery are likely to be more successful in treating it.