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Vulva Cancer

Disease Details

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Description: Vulva cancer is a malignancy that occurs on the external female genital organs, often affecting the labia, clitoris, or vaginal opening.
Type: Vulva cancer is categorized as a type of gynecological cancer.

Regarding genetic transmission, vulvar cancer is generally not considered to be hereditary. Instead, it is more commonly associated with risk factors such as human papillomavirus (HPV) infection, advancing age, and chronic skin conditions like lichen sclerosus. However, in rare cases, genetic syndromes like Cowden syndrome (caused by mutations in the PTEN gene) can increase susceptibility to various cancers, including vulvar cancer.
Signs And Symptoms: The signs and symptoms can include:

Itching, burn, or bleeding on the vulva that does not go away.
Changes in the color of the skin of the vulva, so that it looks redder or whiter than is normal.
Skin changes in the vulva, including what looks like a rash or warts.
Sores, lumps, or ulcers on the vulva that do not go away.
Pain in the pelvis, especially during urination or sex.Typically, a lesion presents in the form of a lump or ulcer on the labia majora and may be associated with itching, irritation, local bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. The labia minora, clitoris, perineum and mons pubis are less commonly involved. Due to modesty or embarrassment, people may put off seeing a doctor.Melanomas tend to display the typical asymmetry, uneven borders and dark discoloration as do melanomas in other parts of the body.
Adenocarcinoma can arise from the Bartholin's gland and present with a painful lump.
Prognosis: Overall, five-year survival rates for vulvar cancer are around 78% but may be affected by individual factors including cancer stage, cancer type, patient age and general medical health. Five-year survival is greater than 90% for patients with stage I lesions but decreases to 20% when pelvic lymph nodes are involved. Lymph node involvement is the most important predictor of prognosis.Prognosis depends on the stage of cancer, which refers the amount and spread of cancer in the body. The stages are broken into four categories. Stage one also called "localized" and is when the cancer is limited to one part of the body. This has the highest survival rate of 59%. When the cancer starts to spread this is referred to "distant" or "regional", this stage usually involves the cancer being spread to the lymph nodes. This survival rate is 29%. The third stage is when the cancer has metastasized and spread throughout the body, this is the lowest survival rate of 6%. When vulvar cancer is caught early that is when the survival rate is at its highest.
Onset: Vulva cancer typically occurs in older women, with the average age at diagnosis being around 65 to 70 years. However, it can occur at any age, though it is rare in younger women. The onset of symptoms can be slow and may include persistent itching, pain, or tenderness of the vulva, changes in vulva skin color, or the presence of a lump or ulcer.
Prevalence: Vulvar cancer is relatively rare compared to other gynecological cancers, accounting for about 0.7% of all female cancer cases. The incidence rate is approximately 2-3 per 100,000 women per year. It is most commonly diagnosed in older women, particularly those over the age of 65.
Epidemiology: Vulvar cancer newly affected about 44,200 people and resulted in 15,200 deaths globally in 2018.Vulvar cancer can be split up into two types. One starts as an infection by human papillomavirus, which leads to vulvar intraepithelial neoplasia (VIN) and potentially on to vulvar cancer. This is most common in younger women, predominantly under the age of 40. The second type is vulvar non-neoplastic epithelial disorders (VNED). This is most common in older women, due to the increased risk for developing cellular atypia which in turn leads to cancer.
Intractability: Vulvar cancer is not generally considered intractable. While it can be serious and challenging to treat, advances in medical treatments such as surgery, radiation therapy, and chemotherapy have improved outcomes for many patients. Early detection and appropriate treatment are key to managing the disease effectively.
Disease Severity: Vulva cancer varies in severity depending on the stage at diagnosis, histological type, and the overall health of the patient. Early stages may be localized to the vulva, with high chances of successful treatment and lower morbidity. Advanced stages can involve significant spread to adjacent structures, lymph nodes, or distant organs, leading to more complex treatments and a higher risk of complications. Early detection improves prognosis, while late-stage diagnosis often involves more aggressive therapies and has a poorer outlook. Regular check-ups and awareness of symptoms are critical for early identification and effective management.
Healthcare Professionals: Disease Ontology ID - DOID:1245
Pathophysiology: Vulvar cancer is a malignancy occurring on the external female genitalia. The pathophysiology typically involves the uncontrolled growth of abnormal cells in the tissues of the vulva, which may be influenced by factors such as HPV infection, chronic skin conditions (like lichen sclerosus), and immune system deficiencies. It may present as squamous cell carcinoma, which is the most common type, or other forms like melanoma, adenocarcinoma, or basal cell carcinoma. Genetic mutations, chronic inflammation, or precursor lesions like vulvar intraepithelial neoplasia (VIN) can all contribute to the development of vulvar cancer.
Carrier Status: Vulvar cancer is not typically associated with a carrier status, as it is not usually caused by a single inherited genetic mutation. Instead, it is more commonly linked to risk factors such as human papillomavirus (HPV) infection, smoking, and certain skin conditions affecting the vulva. In some cases, a familial history of cancer can increase risk, but there is no specific "carrier status" for vulvar cancer akin to those seen in some other hereditary cancers.
Mechanism: Vulva cancer is primarily caused by the uncontrolled growth of malignant cells in the vulvar region. The molecular mechanisms involved are varied and can include:

1. **Human Papillomavirus (HPV) Infection**: High-risk strains of HPV, particularly HPV-16 and HPV-18, are associated with the development of vulvar cancer. The viral oncogenes E6 and E7 from these HPV strains inactivate tumor suppressor proteins p53 and Rb, respectively, leading to unregulated cell proliferation.

2. **Genetic Mutations**: Mutations in specific genes, such as TP53 (which encodes the p53 protein), can impair the cell's ability to regulate the cell cycle and apoptosis, contributing to cancer development. Mutations in other genes like HRAS, PIK3CA, and CDKN2A have also been implicated.

3. **Epigenetic Changes**: Alterations in DNA methylation and histone modification can lead to the silencing of tumor suppressor genes and activation of oncogenes, promoting cancer progression.

4. **Chronic Inflammation**: Conditions like lichen sclerosus, a chronic inflammatory disease of the vulva, can predispose to malignancy by inducing prolonged cellular stress and promoting DNA damage.

5. **Immune Evasion**: Tumor cells in vulvar cancer can evade the immune system by downregulating antigen presentation molecules and secreting immunosuppressive factors, aiding in their proliferation and survival.

Understanding these mechanisms is critical for developing targeted therapies and improving diagnostic markers for vulvar cancer.
Treatment: Surgery is a mainstay of therapy depending on anatomical staging and is usually reserved for cancers that have not spread beyond the vulva. Surgery may involve a wide local excision (excision of the tumor with a safety-margin of healthy tissue, that ensures complete removal of the tumor), radical partial vulvectomy, or radical complete vulvectomy with removal of vulvar tissue, inguinal and femoral lymph nodes. In cases of early vulvar cancer, the surgery may be less extensive and consist of wide excision or a simple vulvectomy. Surgery is significantly more extensive when the cancer has spread to nearby organs such as the urethra, vagina, or rectum. Complications of surgery include wound infection, sexual dysfunction, edema and thrombosis, as well as lymphedema secondary to dissected lymph nodes.Sentinel lymph node (SLN) dissection is the identification of the main lymph node(s) draining the tumor, with the aim of removing as few nodes as possible, decreasing the risk of adverse effects. Location of the sentinel node(s) may require the use of technetium(99m)-labeled nano-colloid, or a combination of technetium and 1% isosulfan blue dye, wherein the combination may reduce the number of women with "'missed"' groin node metastases compared with technetium only.Radiation therapy may be used in more advanced vulvar cancer cases when disease has spread to the lymph nodes and/or pelvis. It may be performed before or after surgery. In early vulvar cancer, primary radiotherapy to the groin results in less morbidity but may be linked with a higher risk of groin recurrence and reduced survival compared to surgery. Chemotherapy is not usually used as primary treatment but may be used in advanced cases with spread to the bones, liver or lungs. It may also be given at a lower dose together with radiation therapy. Checkpoint inhibitors may be given in melanoma of the vulva.There is no significant difference in overall survival or treatment‐related adverse effects in women with locally advanced vulval cancer when comparing primary chemoradiation or neoadjuvant chemoradiation with primary surgery. There is a need for good quality studies comparing various primary treatments.Women with vulvar cancer should have routine follow-up and exams with their oncologist, often every three months for the first 2–3 years after treatment. They should not have routine surveillance imaging to monitor the cancer unless new symptoms appear or tumor markers begin rising. Imaging without these indications is discouraged because it is unlikely to detect a recurrence or improve survival and is associated with its own side effects and financial costs.
Compassionate Use Treatment: Compassionate use, also known as expanded access, allows patients with serious or life-threatening conditions to access investigational drugs outside of clinical trials when no comparable or satisfactory alternative treatments are available. For vulvar cancer, compassionate use might be considered for experimental therapies that are currently undergoing clinical trials but not yet approved by regulatory agencies.

Off-label or experimental treatments for vulvar cancer might include:

1. **Immunotherapy**: Drugs such as pembrolizumab (Keytruda) or nivolumab (Opdivo) are FDA-approved for other types of cancers and may be used off-label for advanced vulvar cancer.

2. **Targeted Therapy**: Agents targeting specific molecular pathways, like EGFR inhibitors (e.g., cetuximab) might be used experimentally.

3. **PARP Inhibitors**: Drugs like olaparib, which are used in ovarian and breast cancer linked to BRCA mutations, may be considered for vulvar cancer patients with similar genetic profiles.

4. **Clinical Trials**: Participation in clinical trials gives patients access to cutting-edge treatments such as novel chemotherapeutic agents, combination therapies, or other innovative approaches not yet widely available.

It is important for patients to discuss these options with their oncology team to determine the most appropriate course of action based on their specific medical condition and treatment history.
Lifestyle Recommendations: For vulva cancer, lifestyle recommendations include:

1. **Avoiding Tobacco**: Refrain from smoking, as tobacco use has been linked to an increased risk of various cancers, including vulvar cancer.
2. **Healthy Diet**: Consume a balanced diet rich in fruits, vegetables, and whole grains while limiting processed foods.
3. **Safe Sex Practices**: Use condoms and limit the number of sexual partners to reduce the risk of human papillomavirus (HPV) infection, which is associated with an increased risk of vulvar cancer.
4. **Regular Exercise**: Engage in regular physical activity to maintain a healthy weight and boost the immune system.
5. **Routine Medical Check-Ups**: Visit a gynecologist regularly for pelvic exams and screenings, which can help detect any abnormalities early.
6. **HPV Vaccination**: Consider getting the HPV vaccine, which protects against the strains of HPV most commonly linked to genital cancers.
7. **Stress Management**: Practice stress-reduction techniques such as mindfulness, yoga, or meditation to improve overall well-being.

Implementing these lifestyle measures can contribute to reducing the risk of developing vulvar cancer and enhance overall health.
Medication: Vulvar cancer treatment typically involves surgery, radiation therapy, and in some cases, chemotherapy. Medications used may include:

1. **Chemotherapy Agents**: Drugs like cisplatin, 5-fluorouracil (5-FU), and mitomycin can be used, particularly for advanced stages or when combined with radiation therapy.

2. **Targeted Therapy**: Drugs that target specific molecules involved in the growth and spread of cancer, like Bevacizumab, are occasionally used.

3. **Immunotherapy**: Pembrolizumab, an immune checkpoint inhibitor, may be used in some advanced cases.

Always consult with an oncologist for a treatment plan tailored to the individual patient's condition.
Repurposable Drugs: Repurposable drugs for vulvar cancer are an area of ongoing research. Some examples of such drugs, which were originally developed for other conditions but have shown potential against vulvar cancer, include:

1. Metformin: Commonly used for type 2 diabetes, metformin has shown anticancer properties in various studies.
2. Statins: Typically used to lower cholesterol, statins have demonstrated potential antiproliferative effects in some cancers.
3. Nonsteroidal anti-inflammatory drugs (NSAIDs) like aspirin and celecoxib: These have been investigated for their potential to reduce cancer risk and progression due to their anti-inflammatory properties.

Each of these drugs requires thorough clinical investigation to confirm their efficacy and safety in the context of vulvar cancer.
Metabolites: For vulvar cancer, metabolites are often examined to understand disease mechanisms and potential therapeutic targets. Key metabolites relevant to vulvar cancer can include those involved in pathways like lipid metabolism, apoptosis, and cellular energetics. Changes in specific metabolites, such as fatty acids, phospholipids, and glucose derivatives, may be indicative of disease presence or progression.

The term "nan" or "not a number" typically indicates missing or undefined data. If you meant "nanomaterials," their role in vulvar cancer is a burgeoning field. Nanomaterials are being explored for their potential in targeted drug delivery, imaging, and diagnostics due to their ability to interact at the molecular level with cancerous cells.

Please clarify if you need more detailed information in a specific area.
Nutraceuticals: Nutraceuticals refer to food-derived products that provide health and medical benefits. For vulvar cancer, there is no conclusive scientific evidence to suggest that nutraceuticals can prevent or treat the disease effectively. Supplements or dietary options like vitamins, minerals, antioxidants, and plant extracts may support overall health or aid in cancer recovery when used alongside conventional treatments, but they should not replace conventional medical therapies. Always consult healthcare professionals before beginning any new supplement regimen.
Peptides: Vulvar cancer treatment involving peptides and nanotechnology is an emerging area of research. Peptides, short chains of amino acids, can be used in targeted therapies to bind specifically to cancer cells, potentially enhancing treatment efficacy and reducing side effects. Nanotechnology involves the use of nanoparticles to deliver drugs or therapeutic agents directly to the cancer cells, improving treatment precision and reducing systemic toxicity. Both areas hold promise for more effective and personalized treatments for vulvar cancer.