Paget’s disease of the vulva is a rare form of skin cancer affecting the vulva, the outer part of the female genitalia. This condition is considered complex due to its often subtle presentation and the challenges associated with its diagnosis and ongoing management. Understanding this disease is important for early identification and effective care.
Understanding Paget’s Disease of the Vulva
Paget’s disease of the vulva is classified as an intraepithelial adenocarcinoma, meaning it originates in glandular cells within the surface layer of the vulvar skin. It is a form of extramammary Paget’s disease (EMPD) that typically progresses slowly.
This disease accounts for approximately 1% to 2% of all vulvar neoplasms. It is more frequently observed in postmenopausal Caucasian women, with a median age of diagnosis around 70 years.
Recognizing the Symptoms
The symptoms of Paget’s disease of the vulva can be non-specific, often mimicking other benign vulvar conditions, which can delay early diagnosis. Common visual characteristics include red, scaly, and itchy patches of skin on the vulva, sometimes with white areas. These lesions can appear similar to eczema or other rashes.
Patients often report persistent itching. Other symptoms include a burning sensation, soreness, irritation, and pain in the affected area. Occasionally, a lump may be present, or there might be bleeding. Some individuals may not experience any symptoms at the time of diagnosis.
Diagnosing the Condition
Diagnosing Paget’s disease of the vulva requires a thorough process, with biopsy being the definitive method for confirmation. The rarity of the disease and its resemblance to other dermatological or inflammatory conditions present significant diagnostic challenges. Conditions like eczema, fungal infections, psoriasis, or lichen sclerosus can have similar appearances, making differential diagnosis crucial.
A punch biopsy or excisional biopsy is performed to obtain a tissue sample for pathological examination. This microscopic analysis identifies the characteristic Paget cells, which are large, pale, and often contain mucin. A gynecologic pathologist confirms the diagnosis. Imaging studies might also be conducted to determine if there is underlying invasive disease or associated internal malignancies.
Treatment Approaches
Surgical excision is the primary treatment for Paget’s disease of the vulva. The goal is a wide local excision with clear margins, meaning the removal of affected tissue along with a surrounding border of healthy tissue, to reduce recurrence risk. Achieving clear margins can be challenging because the disease may extend beyond what is visibly apparent.
Techniques like Mohs micrographic surgery or wide excisions are used to ensure complete removal while preserving healthy tissue. Skin grafting may be necessary following extensive excisions. Non-surgical options, such as topical therapies like imiquimod or 5-fluorouracil, laser therapy, or radiation therapy, may also be considered. These approaches may be used as adjuncts or for recurrent disease, though their effectiveness can vary, and recurrence rates can be high.
Living with Paget’s Disease: Long-Term Management and Outlook
The prognosis for Paget’s disease of the vulva is favorable when the disease is non-invasive, meaning it is confined to the epidermis. However, the disease has a high recurrence rate, with many patients experiencing multiple recurrences regardless of the initial treatment. Recurrence rates of 34% at a median of 3 years have been reported.
Long-term follow-up and surveillance are important due to this high recurrence risk and the potential for associated internal malignancies. Patients are at an increased risk for synchronous (occurring at the same time) or metachronous (occurring at different times) cancers, including colorectal adenocarcinoma, cervical adenocarcinoma, transitional cell carcinoma of the urinary tract, and breast cancer. Regular clinical examinations, including routine screenings such as colonoscopies and mammograms, are recommended to monitor for recurrence and detect any new associated cancers.