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

• Extramammary Paget’s Disease (EMPD) arising in the Scrotum
• Paget Disease - Scrotum
• Scrotal Paget’s Disease

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

• The scrotum is part of the male genitalia that hold the testicles. Paget’s Disease of the Scrotum is an extremely rare form of Paget’s disease, with an extramammary origin. In addition to the scrotum, Extramammary Paget’s Disease (EMPD) can also arise in the groin (vulva or penis) and perineum region
• Although Paget’s disease is associated with malignant tumors, thus far, there are no reports of Paget’s Disease of the Scrotum being associated with other malignancies
• Some risk factors for developing for Scrotal Paget’s Disease are advanced age, obesity, radiation exposure, being a Caucasian, etc. However, the exact causative factors of the condition are not yet known
• The presence of a lesion in the scrotal sac is the most typical symptom associated with the condition. Patients often report a burning and itchy sensation. Ulceration of the lesion is also a possibility
• Surgery, by far, is the best option for treatment of Paget’s Disease of the Scrotum. The prognosis for the condition is considered good when diagnosed early and treated promptly
• Since the cause of Paget’s Disease of the Scrotum is not known, preventive measures have not been identified. However, leading a healthy lifestyle with moderate physical activity, not smoking and moderate consumption of alcohol are some ways to reduce possibility of contracting the cancer

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

• Paget’s Disease of the Scrotum is a rare condition and is typically observed in older men, with a mean age at presentation of 71 years
• The condition affects people of all geographic regions around the world
• It has been reported that Caucasians bear a higher risk of being diagnosed with Paget’s Disease of the Scrotum

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

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

• Advanced age
• Exposure to radiation
• Obesity
• Race, since it has been reported that being Caucasian increases the risk of one developing the condition

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 Scrotum? (Etiology)

• The cause of Paget’s Disease of the Scrotum is not known at this time
• Some gene mutations are being researched upon to understand the basis of Paget’s disease

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

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

• Lesion on the skin of scrotal sac
• Inflamed-appearing rash
• Rash that itches and burns
• Eczema-like appearance of the lesion (scaly lesions)
• Ulceration of the lesion
• Bleeding
• Increased sensitivity in the scrotal region
• Pain

Initially, Paget’s Disease of the Scrotum might appear like other superficial skin conditions.

How is Paget’s Disease of the Scrotum Diagnosed?

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

• A physical examination and an assessment of a patient’s medical history
• Ultrasound scan
• Computed tomography (CT) and magnetic resonance imaging (MRI) scans
• Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

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 Scrotum?

The following may be some complications of Paget’s Disease of the Scrotum:

• Bleeding and ulceration might lead to bacterial and fungal infections
• Pain/discomfort during intercourse
• Stress, anxiety
• Tumor metastasis - to the lymph nodes of the groin and other regions of the body
• Recurrence of the condition even after treatment - Paget’s disease of the Scrotum is reported to recur and could become chronic
• Side-effects of chemotherapy and radiation therapy

How is Paget’s Disease of the Scrotum Treated?

The best treatment option for Paget’s Disease of the Scrotum is considered to be surgery.

• In majority of 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 Scrotal Paget’s Disease. The surgical procedures performed could include:
  • Scrotal reduction, or partial removal of scrotum
  • Complete removal of scrotum: Additionally, sometimes, local lymph nodes may also be removed with bilateral inguinal and femoral lymphadenectomy
  • Partial pelvic exenteration may be performed if the tumor is large. 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
• For metastasized tumors (in rare cases),  a combination of chemotherapy, radiation therapy, and invasive procedures may be used
• In some cases, non-surgical treatment methods may be employed, which include radiotherapy, chemotherapy, laser therapy (using CO2), and photodynamic therapy
• Counseling to help with stress and anxiety
• Post-operative care - to allow the wound to heal
• Follow-up care with regular screening and check-ups 

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

Since the exact cause of Paget’s Disease of the Scrotum is not known, no preventive methods have thus far been reported for this condition. Nevertheless, maintaining a healthy lifestyle could help one avoid/delay the onset of disease. The following tips might be helpful:

• Maintaining healthy lifestyle habits
• Leading an active life
• Eating a healthy diet
• Limiting alcohol and smoking

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

• With early diagnosis and prompt treatment, the prognosis for Paget’s Disease of the Scrotum is reported to be good
• The stage of the tumor and the extent of spread might determine the final outcome

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

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