What are the other Names for this Condition? (Also known as/Synonyms)

• Extramammary Paget’s Disease (EMPD) arising in the Vulva
• Paget Disease - Vulva
• Vulvar Paget’s Disease

What is Paget’s Disease of the Vulva? (Definition/Background Information)

• Paget’s Disease of the Vulva is a very rare form of Paget’s disease that arises from a location outside the breast (extramammary site). In addition to the vulva, Extramammary Paget’s Disease (EMPD) can also arise in the groin (penis and scrotum) and perineum region).
• The condition may be associated with other malignant tumors. It is researched that approximately 1 in 10 individuals with Paget’s disease have a malignancy elsewhere in the body
• Postmenopausal women have the greatest risk for Vulvar Paget’s Disease. The other risk factors associated with the condition may include radiation exposure and obesity
• The exact cause of Paget’s Disease of the Vulva is not yet known. The lesion is a well-characterized growth, often having burning and itchy sensation that can lead to ulcer formation
• Paget’s Disease of the Vulva is best treated with surgery. The prognosis depends on how far the underlying cancer has spread to other parts of the body

Who gets Paget’s Disease of the Vulva? (Age and Sex Distribution)

• Paget’s Disease of the Vulva is most commonly seen in women over the age of 51 years (post-menopausal stage)
• Paget’s Disease of the Vulva does not affect any specific race or ethnic group. Nevertheless, Caucasians are observed to have a higher incidence of the condition

What are the Risk Factors for Paget’s Disease of the Vulva? (Predisposing Factors)

Risk factors associated with Paget’s Disease of the Vulva are:

• Age: The risk increases with advancing age in women; those who are above the menopausal age range of 48-55 years have a high risk
• Genetic predisposition: Defective genes in the body can make an individual susceptible to Paget’s Disease of the Vulva
• Radiation exposure: Radiation exposure for the treatment of some other cancer elevates the risk
• The risk of Vulvar Paget’s Disease is increased with obesity
• Hormone replacement: Intake of estrogen after menopause increases the risk for the condition
• Race: Caucasian women are at a higher risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Paget’s Disease of the Vulva? (Etiology)

• The cause of Paget’s Disease of the Vulva is generally unknown. It may arise from an underlying cancer
• Certain genetic mutations have been suspected, but these have not been well-characterized

What are the Signs and Symptoms of Paget’s Disease of the Vulva?

The signs and symptoms manifested by Paget’s Disease of the Vulva include:

• The presence of a pink or red lesion on the vulva
• Rash and itchiness
• The presence of scaly skin on the lesions
• Formation of ulcers in the infected area
• Bleeding in the affected area
• Burning sensation
• Increased sensitivity
• Pain and discomfort

How is Paget’s Disease of the Vulva Diagnosed?

Diagnosis of the Paget’s Disease of the Vulva includes:

• Complete physical examination and detailed medical history evaluation
• Radiological studies, such as ultrasound scan, CT and MRI scans, to determine the extent of tumor spread
• Tissue biopsy:  A tissue sample is sent to a pathology lab for examination under a microscope. The pathologist examines the biopsy sample that can help make a definitive diagnosis. The pathologist may make additional studies to arrive at a conclusion

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Paget’s Disease of the Vulva?

The complications of Paget’s Disease of the Vulva may include:

• Secondary infections of bacteria and fungus due to bleeding and ulceration
• Painful or uncomfortable sexual intercourse
• The tumor can metastasize to the lymph nodes of the groin and other regions of the body
• Severe emotional and psychological stress
• Recurrence of the condition following treatment: Paget’s disease can be chronic with multiple recurrences. Some studies have shown the recurrence rate to be up to 50%
• Complications that arise from cancer therapy (such as due to chemotherapy or radiation therapy)

How is Paget’s Disease of the Vulva Treated?

Paget’s Disease of the Vulva is best treated using surgical procedures.

• In most cases, a wide surgical excision and removal of the entire tumor is the preferred treatment option. This may be followed by radiation therapy and/or chemotherapy
• The surgical removal of the tumor depends on the size and stage of the Vulvar Paget’s Disease. The surgical procedures performed could include:
  • Hemivulvectomy, which is partial removal of vulva
  • Total vulvectomy, which is complete removal of vulva. Sometimes, local lymph nodes may also be removed using total vulvectomy, with bilateral inguinal and femoral
  • If the tumor size is large, then partial pelvic exenteration may be performed. During this procedure, all the cancer tissue and surrounding pelvic tissue is removed to decrease the bulk of the tumor. This is often followed by radiation therapy and chemotherapy
• If the tumor has metastasized (in rare cases), then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
• Undertaking treatment of any associated cancers
• In some cases, non-surgical treatment methods may be employed, which include radiotherapy, chemotherapy, laser therapy (using CO2), and photodynamic therapy
• Some women may require reassurance to help with stress and anxiety
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important and encouraged

How can Paget’s Disease of the Vulva be Prevented?

Currently, no specific preventative methods are known for Paget’s Disease of the Vulva. However, adopting certain general preventative measures, such as the following, may reduce the risk of cancer:

• Healthy lifestyle habits
• Mild exercises
• Eating a healthy diet
• Limiting alcohol and smoking

What is the Prognosis of Paget’s Disease of the Vulva? (Outcomes/Resolutions)

• The prognosis for Paget’s Disease of the Vulva is generally good with treatment (surgical excision)
• Although, it also depends on the stage of the underlying condition and associated malignancies, if any

Additional and Relevant Useful Information for Paget’s Disease of the Vulva:

Extramammary Paget’s disease has been sub-classified into the following categories:

• Type 1a – Primary Cutaneous Extramammary Paget’s Disease: In this type, the Paget’s disease occurs in the epidermis of skin, or it involves the sweat glands associated with the skin (namely the apocrine, eccrine, or sebaceous sweat glands)
• Type 1b – Primary Cutaneous Extramammary Paget’s Disease: In this type, the Paget’s disease is associated with either adenocarcinoma in situ or with invasive Paget’s disease
• Type 2 – Extramammary Paget’s Disease: In this type, the Paget’s disease occurs in association with underlying adenocarcinoma of anus or rectum
• Type 3 – Extramammary Paget’s Disease: In this type, the Paget’s disease occurs in association with the underlying adenocarcinoma of anus or rectum, and underlying bladder adenocarcinoma