OVERVIEW

What is vulvar malignant melanoma?

Vulvar malignant melanoma is a malignant tumor of the vulva, with an unclear etiology, usually arising from the malignant transformation of vulvar pigmented nevi. Patients commonly experience symptoms such as vulvar itching, local bleeding, pigment changes, and pain. Lesions often occur on the labia majora, labia minora, clitoris, and around the urethra, typically presenting as nodular or mole-like growths, usually dark brown or blue-black, and may be accompanied by ulcers ^[1]. Some patients may have lesions without melanocytes, showing no pigmentation symptoms ^[21].

Due to its concealed location and inconspicuous early symptoms, diagnosis and treatment are often delayed. This tumor is highly malignant with a poor prognosis, prone to recurrence and metastasis. The primary treatment is surgical resection, which may be combined with radiotherapy, chemotherapy, and drug therapy ^[2].

Is vulvar malignant melanoma common?

No, it is uncommon.

Vulvar malignant melanoma predominantly affects postmenopausal women aged 54–76, with a higher incidence among white women. The incidence rate is approximately (0.48–1.4) per million women, meaning roughly one in a million women may develop the disease ^[2].

SYMPTOMS

What are the common symptoms of vulvar malignant melanoma?

The disease mainly presents as vulvar masses, local pain, bleeding, and itching. A small number of patients may have no obvious symptoms. Vulvar malignant melanoma typically appears as nodules or spots with asymmetrical edges and irregular pigmentation, which may be accompanied by ulcers. Most lesions are pigmented, appearing brown, black, blue, pink, etc., while a few may lack pigmentation ^[3].

What are the complications of vulvar malignant melanoma?

Tumor metastasis: This disease has a high degree of malignancy. The tumor may directly grow into adjacent organs (such as the urethra, vagina, anus, etc.) or spread to lymph nodes in the groin or pelvic region, leading to symptoms such as difficulty urinating, bleeding during intercourse, difficulty defecating, and abdominal pain ^[1].

CAUSES

What are the causes of vulvar malignant melanoma?

The exact cause of this disease is unclear, but it may be related to the following factors ^[1,4]:

• Age: The immune function of middle-aged and elderly women gradually declines, and postmenopausal endocrine function is prone to imbalance, increasing the likelihood of malignant melanoma.
• Viral infections: Such as human papillomavirus (HPV) infection, which can cause skin lesions and malignant tumors in the vulva and other areas.
• Genetics: Malignant melanoma shows familial clustering, and studies indicate its occurrence is related to genes.
• Environment: Intense sun exposure or prolonged time in the sun (e.g., extended sunbathing at the beach without sunscreen) and UV stimulation increase the risk of malignant melanoma in various skin areas.
• Race: Caucasians have a higher likelihood of developing this disease than other races.

Which women are more likely to develop vulvar malignant melanoma?

• Postmenopausal women;
• Women infected with human papillomavirus (HPV) or other sexually transmitted diseases;
• Women with a family history of melanoma or a personal history of melanoma;
• Women with prolonged sun exposure, especially those of Caucasian descent.

Is vulvar malignant melanoma contagious?

No.

Is vulvar malignant melanoma hereditary?

Possibly. About 3%–10% of malignant melanoma patients have a family history, though the incidence is lower among Asians ^[4].

DIAGNOSIS

How to Diagnose Vulvar Malignant Melanoma? What Tests Are Needed?

The following tests are primarily required ^[3]:

• Gynecological Examination: The doctor examines and palpates the vulvar area, including surrounding tissues and regional lymph nodes. Patients should prepare mentally and cooperate with the doctor.
• Dermoscopy: Commonly used for diagnosing various skin conditions, it can assist in evaluating vulvar pigmented lesions.
• Imaging Tests: Including inguinal lymph node ultrasound, contrast-enhanced CT or MRI of the abdomen and pelvis, and whole-body bone scans. These help assess the extent of tumor metastasis and staging.
• Lesion Biopsy: The lesion is usually completely excised. For larger lesions, a partial biopsy may be performed to confirm the diagnosis.

What Diseases Should Vulvar Malignant Melanoma Be Distinguished From?

It must be differentiated from vulvar pigmented nevi. Vulvar melanoma often presents with pigmentation, appearing primarily black but also brown, red, blue, or pink. Its edges are irregular, possibly jagged and slightly raised. Early stages may show no obvious pain or itching. In contrast, vulvar nevi usually have a uniform color and are round or oval. Despite clinical differences, vigilance for malignant transformation of nevi is essential. A tissue biopsy is typically used to confirm the diagnosis ^[3].

TREATMENT

Which department should I visit for vulvar malignant melanoma?

Gynecology, Oncology.

How is vulvar malignant melanoma treated?

Treatment typically involves surgery, supplemented by radiotherapy, chemotherapy, and medication ^[3]:

• Surgical treatment: Usually involves wide local excision of the tumor, and radical resection if necessary. If regional lymph node metastasis is present, regional lymph node dissection is also performed during surgery. The procedure is typically performed under local anesthesia, but general anesthesia may be chosen depending on the condition. The duration of surgery and postoperative recovery time depend on factors such as tumor size and extent of spread. Surgery usually takes less than two hours, and patients may require about a month to recover.

• Radiotherapy: Helps control local lesions and reduce recurrence rates. It is generally used for advanced patients who cannot undergo surgery or those with postoperative recurrence or metastasis. Currently, heavy particle radiotherapy has shown significant efficacy in treating vulvar cancer.

• Medication: Includes immune checkpoint inhibitors and targeted therapy drugs.

  1. Immune checkpoint inhibitors: Block the inhibitory effects of tumor cells on immune cells. Commonly used drugs include nivolumab, pembrolizumab, and ipilimumab. Side effects after injection may include loss of appetite, dizziness, headache, cough, endocrine disorders, rash, hepatitis, and neutropenia. These drugs are contraindicated for patients with allergies.

  2. Targeted therapy drugs: Destroy specific structures of tumor cells, leading to their death. Commonly used drugs include imatinib, dabrafenib combined with trametinib, and axitinib. Adverse reactions may include nausea, vomiting, diarrhea, rash, fever, loss of appetite, headache, palpitations, joint and muscle pain, blood pressure changes, and liver damage. These drugs are contraindicated for patients with severe liver dysfunction, heart failure, bone marrow suppression, or allergies.

• Chemotherapy: Uses chemical agents to kill some tumor cells. It is usually considered when drug resistance occurs in medication therapy. Advanced patients may undergo radiotherapy combined with chemotherapy.

DIET & LIFESTYLE

What should patients with vulvar malignant melanoma pay attention to in their diet?

• Postoperative patients are advised to consume liquid foods, such as rice porridge, to reduce bowel movements. Avoid spicy and irritating foods to prevent affecting wound recovery;
• Quit smoking and drinking to avoid interfering with the condition;
• Maintain a light daily diet, eat more fresh fruits and vegetables to supplement vitamins and promote physical recovery;
• Consume high-quality proteins, such as beef, lean meat, loach, and eggs, to enhance immunity.

What should patients with vulvar malignant melanoma pay attention to in daily life?

• Postoperative patients should pay attention to wound hygiene and avoid squeezing the urinary catheter;
• Maintain a regular schedule, avoid staying up late, and refrain from strenuous exercise after surgery;
• Follow medical advice and take medication on time and in the prescribed dosage;
• If adverse reactions such as headache or vomiting occur after medication, inform the doctor promptly to adjust the treatment plan;
• Wear loose underwear daily and maintain vulvar hygiene.

Is follow-up examination necessary for vulvar malignant melanoma?

Yes, lifelong follow-up examinations are required after surgery for vulvar malignant melanoma. The typical follow-up schedule is: one month after surgery, then every three months for the first two years, every six months after two years, and annually after five years ^[5].

The main follow-up examinations include gynecological examinations and imaging tests (such as ultrasound, CT scans, MRI, etc.). Gynecological tumor marker tests may be added if necessary.

PREVENTION

Can Vulvar Malignant Melanoma Be Prevented? How to Prevent It?

• Avoid excessive sun exposure and always use sun protection;
• Quit smoking, maintain a balanced diet;
• Engage in regular physical activities like jogging or walking to boost immunity;
• Maintain vulvar hygiene and practice safe sex with condoms;
• Consider HPV vaccination to prevent HPV-related cancers;
• Postmenopausal women and those with a personal or family history of melanoma should undergo regular gynecological check-ups.