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

• Extramammary Paget’s Disease (EMPD) arising in the Penis
• Paget Disease - Penis
• Penile Paget’s Disease

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

• Paget’s Disease of the Penis is a rare type of Paget’s disease that affects extramammary sites, or parts of the body outside of the breast. The condition causes lesions on the penis shaft. In addition to the penis, Extramammary Paget’s Disease (EMPD) can also arise in the groin (vulva or scrotum) and perineum region
• The disease may be linked with other malignant cancers. Studies show that about 10% of the individuals with Paget’s disease also show malignancies in separate areas of the body. In fact, research shows that isolated cases of Paget’s Disease of the Penis rarely occur
• Men of advanced age are the most at risk for Penile Paget’s Disease. In addition to age, radiation exposure and obesity greatly increase an individual’s chance of acquiring the condition
• The underlying cause for Paget’s Disease of the Penis is under investigation. The lesion associated with Paget’s Disease of the Penis grows in a well-characterized manner, is often accompanied by burning and itching, and may eventually give rise to ulcers
• Surgery is the most effective course of action in dealing with Paget’s Disease of the Penis. The prognosis depends on how advanced the disease has become and the extent to which it has invaded other body sites

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

• Paget’s Disease of the Penis is rare and confined largely to elderly males between the ages of 55 and 84 years. On an average, individuals are 71 years old when they have developed the disease
• Paget’s Disease of the Penis affects people all over the world, with no preference for race or ethnicity except for Caucasians, who possess a greater risk for the condition

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

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

• Age: The risk increases with advancing age in men; older men have a higher risk
• Genetic predisposition: Defective genes in the body can make an individual susceptible to Paget’s Disease of the Penis
• Radiation exposure: Radiation exposure for the treatment of some other cancer elevates the risk
• The risk of Penile Paget’s Disease is increased with obesity
• Race: Caucasian men 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 Penis? (Etiology)

• The cause of Paget’s Disease of the Penis is under investigation
• One potential cause for the disease is genetic alterations, but further research needs to be done before definitive conclusions are reached

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

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

• The presence of a skin lesion or patch in the penile shaft
• Rash and itchiness, redness
• The presence of scaly skin on the lesions; the condition may resemble eczema
• Formation of ulcers in the infected area
• Bleeding in the affected area
• Burning sensation
• Increased sensitivity
• Pain and discomfort

The condition may appear like other superficial skin conditions.

How is Paget’s Disease of the Penis Diagnosed?

Diagnosis of the Paget’s Disease of the Penis 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 Penis?

The complications of Paget’s Disease of the Penis 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)
• Complications due to the presence of other malignancies

How is Paget’s Disease of the Penis Treated?

Surgery is the most effective method for treating Paget’s Disease of the Penis.

• Often, this requires the removal of a large area of tumor, which often combined with radiation therapy and/or chemotherapy.
• The procedures necessitated to remove the tumor caused by Penile Paget’s disease depend on the extent to which the disease has progressed. These procedures may include:
  • Partial penectomy, which is the partial removal of the penis
  • Complete penectomy, which is the total removal of the penis. Sometimes, local lymph nodes may also be removed with bilateral inguinal and femoral lymphadenectomy
  • 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
• In the rare event that the tumor has metastasized (spread), chemotherapy, radiation therapy, and invasive procedures may need to be combined to create an effective treatment
• Radiotherapy, chemotherapy, laser therapy using CO2, photodynamic therapy, and other non-surgical procedures may be effective in certain instances
• Treatment would also have to be performed for any associated disorders/cancers
• Certain individuals may require additional help in dealing with the stress and anxiety that often results from the disorder and its treatment
• Remaining active is a component of post-operative care, which is very important to accelerate healing

It is recommended to follow up with regular check-ups.

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

Though nothing can prevent Paget’s Disease of the Penis with certainty, enacting the following general guidelines may decrease the chance for acquiring the condition:

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

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

• The prognosis for Paget’s Disease of the Penis is generally good with treatment (surgical excision)
• However, it also depends on the stage of the underlying condition and the extent of spread

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

Extramammary Paget’s disease falls into the following categories:

• Type 1a – Primary Cutaneous Extramammary Paget’s Disease: Affects the skin’s epidermis or sweat glands (namely the apocrine, eccrine, or sebaceous sweat glands)
• Type 1b – Primary Cutaneous Extramammary Paget’s Disease: Associated with either adenocarcinoma in situ or with invasive Paget’s disease
• Type 2 – Extramammary Paget’s Disease: Associated with underlying adenocarcinoma of the anus or rectum
• Type 3 – Extramammary Paget’s Disease: Associated with underlying adenocarcinoma of anus or rectum, in addition to underlying bladder adenocarcinoma