Decoding Vulvar Melanoma : A Comprehensive Guide to Understanding and Managing the Disease

1. What is Vulvar Melanoma

   Vulvar melanoma is an aggressive form of skin cancer that originates in the melanocytes, cells of the vulva that produce pigment. Vulva is the external part of female genitalia including labia majora, labia minora, clitoris, and vaginal opening.⁽¹⁾ Melanoma arises when the melanocytes undergo malignant transformation and start growing uncontrollably.

   Vulvar melanoma is relatively uncommon compared to other types of skin cancers, such as melanomas occurring in sun-exposed areas. It accounts for 5% of all vulvar cancers, that affect the mucous surface of the structures that make up the vulva.⁽²⁾ About 1% of all females with melanomas have vulvar melanoma.⁽³⁾ The rarity of this cancer delays diagnosis and emphasizes the importance of understanding its clinical presentation, risk factors, and appropriate management strategies.

2. Importance of Understanding and Awareness of Vulvar Melanoma

   Having an understanding and awareness about vulvar melanoma is crucial for several reasons: 

   • Increased awareness can be helpful to the individuals to recognize potential symptoms, leading to early medical consultation and intervention. This can improve the chances of successful treatment and better outcomes.
   • Knowledge of risk factors allows for proactive measures to reduce the likelihood of developing the disease.
   • It can help break down stigmas and encourage open conversation about vulvar health, facilitating early detection and medical consultation.
   • Awareness fosters a supportive environment that encourages empathy, reduces isolation, and promotes mental well-being.

   Understanding and awareness are pivotal in addressing vulvar melanoma by promoting early detection, reducing stigma, facilitating open discussions, educating healthcare professionals, supporting affected individuals, and driving public health initiatives and research efforts.

The etiology of vulvar melanoma involves a combination of genetic, environmental, and hormonal factors. The exact mechanism is not fully understood, the risk factors identified include: 

• Genetic Predisposition: Individuals with a family history of melanoma or a history of genetic mutations associated with melanoma are at an increased risk of developing vulvar melanoma.⁽³⁾
• UV Exposure: Vulvar skin may not be frequently exposed to the sun still UV exposure may contribute to the development of vulvar melanoma.⁽⁴⁾
• Age and Gender: Vulvar melanoma occurs in postmenopausal women, as the risk increases with age.⁽⁴⁾ However, rarely, it can affect women of all age groups.
• Hormonal Influences: Hormonal changes occurring during pregnancy or the use of hormonal therapies, may be associated with an increased risk of vulvar melanoma.⁽⁵⁾
• Previous History: Individuals with a history of melanoma in other parts of the body are more at risk of developing vulvar melanoma.
• Immunosuppression: A weakened immune system, either due to medical conditions or immunosuppressive medications, may contribute to an elevated risk of melanoma development.
• Chronic Inflammation: Conditions causing chronic inflammation in the vulvar region such as lichen sclerosus have been associated with an increased risk of vulvar melanoma.⁽⁶⁾
• Smoking: Studies suggest that smoking may be a risk factor for vulvar melanoma, although the association is not fully understood.⁽⁷⁾
• Human Papillomavirus (HPV) Infection: Ongoing research is exploring the potential link between HPV infection and vulvar melanoma, but the relationship is not well-established.

The clinical presentation may vary and the symptoms may overlap with other benign conditions. Recognizing these signs and symptoms is essential for early detection and prompt medical intervention.

The clinical presentation includes: 

• Painless asymptomatic lesions on the vulva. This absence of symptoms may contribute to delayed diagnosis.
• Changes in pigment on vulvar skin. There may be hyperpigmented or hypopigmented lesions. These may resemble moles and freckles may exhibit irregular borders and uneven coloring.
• Raised nodules or lumps in the vulva, which may vary in size. The nodules may be firm and irregularly shaped and may exhibit a range of colors including brown, black, red, or blue.
• Ulceration of lesion may occur leading to open sores on the vulvar skin. Bleeding oozing, or crusting of the lesions can be observed especially as the disease progresses.
• Itching and pain in the affected areas. Discomfort may be present even in the absence of visible lesions, emphasizing the need for attention.
• Presence of unusual discharge
• Lesions may vary in size, with larger lesions often associated with more advanced stages of the disease. The surface of the lesion may be irregular with areas of elevation, depression, or ulceration.
• Satellite lesions (nodules of tumor more than 0.5mm from the primary lesion), and smaller melanoma growths near the primary tumor may be observed.⁽⁸⁾ In advanced stages, vulvar melanoma may spread to nearby lymph nodes, leading to palpable lymph node enlargement.

Self-examination is important for individuals at risk of vulvar melanoma. Prompt medical attention is needed to notice any concerning changes.

Diagnosing vulvar melanoma involves a combination of clinical evaluation, imaging studies, and pathological examination. Timely and accurate diagnosis is important for determining the appropriate treatment and management strategies. 

• Physical Examination: A thorough physical examination is done focusing on the vulvar region. The appearance, size, color, and texture of any lesion as well as the associated symptoms are assessed. The nearby lymph nodes are also examined.
• Biopsy: Biopsy is a definitive diagnostic procedure. It is performed by taking a small tissue sample from the suspicious lesion and sending it to the laboratory for examination. It helps in confirming the presence of melanoma and providing information about the extent of invasion.
• Histopathological Examination: The biopsy specimen is sent for histopathological examination. The pathologist analyses the tissue under a microscope to assess the characteristics of melanoma cells including their size, shape, and appearance.
• Imaging Studies: Ultrasound, CT scan, and MRI scans can help assess the extent of the disease and identify potential metastasis.
• Staging: Staging is a crucial aspect of the diagnostic process as it helps in determining the extent of the disease.
• Specialized Testing: Additional tests may be conducted to assess specific characteristics of melanoma cells. Immunohistochemistry is performed to detect specific proteins associated with melanoma cells.

Due to the complexity of vulvar melanoma, a multidisciplinary approach involving gynecologists, dermatologists, pathologists, oncologists, and other specialists is essential for comprehensive care and treatment planning.

The treatment of vulvar melanoma involves a combination of surgical intervention, adjuvant therapies, and in some cases palliative care. The choice of treatment depends on the stage of disease, the extent of tumor invasion, and the overall health of the patient.

Treatment strategies include: 

• Surgery: The primary treatment of vulvar melanoma involves surgical removal of a tumor with a wide margin surrounding health tissue to reduce the risk of recurrence.⁽²⁾ The extent of excision depends on the size and location of the lesion.

If there is lymph node involvement, surgical removal of the affected lymph node may be performed.

• Adjuvant Therapies: Immunotherapy involving immunotherapeutic agents such as immune checkpoint inhibitors may be used to enhance the body’s immune response against melanoma cells. These therapies are particularly relevant in cases of metastatic disease.

Targeted therapies may be considered for vulvar melanomas with specific genetic mutations. Drugs like vemurafenib or dabrafenib, often in combination with MEK inhibitors, can target these mutations.

• Chemotherapy: In the case of advanced and metastatic vulva melanoma, systemic chemotherapy is used. However, it is not considered an effective option for those with vulvar melanoma.⁽³⁾
• Radiation Therapy: Radiation therapy is given as an adjuvant treatment following surgery to target any remaining cancer cells or as a palliative care to relieve symptoms.
• Palliative Care: In case of advanced stages of disease where curative options are limited, palliative care is given focusing on alleviating symptoms, improving quality of life, and providing emotional and psychological support.

Regular follow-up appointments are important to monitor the signs and symptoms of recurrence or metastasis. Imaging studies and physical examinations may be conducted to assess the effectiveness of treatment and detect any potential relapse.

Vulvar melanoma is a rare and aggressive form of skin cancer originating from the melanocytes of the vulva. Early detection is important to improve the outcome for individuals with vulvar melanoma. The clinical presentation may vary and it has a resemblance to other benign conditions. Regular self-examination and medical check-ups are therefore important. Recognizing changes in pigmentation, nodules, ulceration, and other symptoms is crucial for prompt diagnosis and intervention.

Once diagnosed, surgical removal of the tumor is done. Additional treatments may be recommended, but they are not very effective.

A collaborative effort of healthcare professionals, researchers, and the public is needed for a better understanding of vulvar melanoma and to improve its treatment options. By combining knowledge, awareness, and proactive healthcare measures, significant strides can be made in managing and ultimately preventing the impact of vulvar melanoma on individuals and communities.