Vulva is the outer part of the female genital tract. It consists of two lips called the labia majora and labia minora. The vagina and urethra open into the vulva. The vulva also contains the clitoris.
Vulva cancer is cancer that has started in the vulva. It is rare cancer and usually presents after the age of 65. vulva cancers grow over a period of time. The normal cells of the vulva region change into a pre-cancerous condition called VIN (vulva intraepithelial neoplasia) before further change turns the cells into cancer. Treatment of VIN promptly reduces the risk of vulva cancer.
Vulva cancer mainly occurs around the labia majora and minora but can also occur in other areas of the vulva including the perineum which is the area between the vagina and anus.
Cancer of the vulva has some risk factors which are listed below. A risk factor is one which increases the risk of getting a cancer.
HPV
HPV stands for Human Papilloma Virus. This is a virus that can lead to HPV infection. This is a sexually transmitted infection and is very common. HPV infection is a risk factor for development of vulva intraepithelial neoplasia(VIN), which is a pre-cancerous condition, and vulva cancer. HPV infection can also cause cervical, vaginal, anal and throat cancers. It is important to note that only a small proportion of people infected with HPV virus go on to develop these cancers. Vaccination against HPV infection is now available.
Smoking
Smoking is a known risk factor for the development of vulva Cancer.
Other Conditions
Diseases of the Vulva such as vulva dystrophy and lichen sclerosis can be risk factors for the development of vulva cancer. Diseases which suppress the immune system such as HIV and AIDS can be risk factors.
vulva cancer can produce a range of symptoms which are listed below.
Common symptoms associated with vulva Cancer include the presence of an ulcer or a lump in the vulva region such as labia majora, labia minora, clitoris or perineum. The mass can be hard or fleshy. A lump felt in the groin region may also be a presenting symptom in vulva cancer. Sometimes, a flat and thickened area may be the presenting feature.
There can be associated symptoms such as bleeding, itching in the area or discomfort with passing urine or motion. The symptoms of itching are also present in non-cancerous conditions such as vulva dystrophy or other skin conditions.
Patients with more advanced disease may present with ulceration in other areas such as the groin, loss of appetite and weight loss.
Presence of any of these above symptoms should alert you to visit the doctor for examination and further tests.
Examination
A thorough examination by a doctor to look for abnormalities on the vulva is done if there are symptoms or suggestions of a vulva cancer. The examination is aided by a magnifying glass or a colposcope. A biopsy may be taken if any suspicious areas are seen.
The biopsy can show a vulva intraepithelial neoplasia (VIN) or a vulva cancer or a benign condition. If vulva cancer is diagnosed on biopsy, further tests will be needed to complete the staging process.
CT Scan or PET-CT scan
A CT scan is a scan that is done to get detailed images of the inside of the body. A CT scan uses x-rays to get these images. A PET-CT is done in place of a CT scan and can give more detailed information when looking for the presence of cancer spread to other parts of the body. PET-CT is a CT scan but with and additional radioactive dye called FdG injected into the patient before the scan is done. Either of these scans can be used.
A cancer is given a stage based on its size, area of spread from its site of origin and involvement of other structures around it. Staging can be given according to the TNM classification or the number staging system. The number system stages all cancers from stage 1 to stage 4 with stage 1 being early cancer and stage 4 being advanced cancer. Knowing the stage of the cancer helps the doctors to decide on the most appropriate treatment.
Cancer of the Vulva is also staged according to the TNM and number staging system and is listed below.
TNM Staging
TNM stands for tumour node and metastases
T Staging
| T stage | FIGO stage number | Description of stage |
|---|---|---|
| Tis | Carcinoma in situ (pre invasive cancer) | |
| T1 | 1 | Tumour confined to vulva |
| T2 | 2 | Tumour of any size extending into adjacent structures |
| T2 | 3 | Tumour of any size with or without extension into adjacent structures And involvement of inguinal lymph nodes |
| T3 | 3a | Tumour involves outer layer of uterus or fallopian tubes or ovaries |
| T3b | 4a | Tumour of any size with further extension than T2 or involvement of bladder, rectum or pelvic bone |
| 4b | Any distant spread including involvement of pelvic lymph nodes |
N Staging
| N Stage | FIGO stage number | |
|---|---|---|
| N0 | No involvement of lymph nodes | |
| N1 | 3a | 1 to 2 lymph nodes less than 5mm or one lymph node more than 5mm |
| N2 | 3b-3c | 2 or more nodes more than 5mm or three or more nodes less than 5mm or nodes with spread outside them |
| N3 | 4a | Lymph nodes that are fixed or ulcer like |
M Stage
| M0 | No distant spread of cancer |
| M1 | Presence of distant metastases which includes positive pelvic lymph nodes (cancer in these nodes) |
The various options used to treat vulva cancer include surgery, radiotherapy and chemotherapy
Surgery
Surgery forms an important part of management of cancer of the Vulva. The type of surgery used depends on the stage of disease at diagnosis.
Wide excision
This is a type of surgery used for very early vulva cancer (stage 1A). Here the cancer along with a good margin of normal vulva is removed. vulva cancer can normally spread to lymph nodes in the groin and upper part of the thigh, but in stage 1A vulva cancer the risk of this spread is low. Therefore, lymph nodes are not removed and only a wide excision is adequate.
Wide excision is also used as a treatment option for vulva intraepithelial neoplasia (VIN), which is a precancerous condition. Here excision is done of the abnormal area along with a margin of normal vulva around it.
Radical Vulvectomy
This is a surgical procedure where the entire vulva is removed along with lymph nodes either on one side (groin and upper thigh) or both sides. The extent of surgery depends on the extent and spread of disease in the vulva and the lymph nodes. In some situations, a partial vulvectomy or a hemi(half) vulvectomy may be adequate. Some centres may do a procedure called sentinel node biopsy prior to doing a lymph node dissection(removal).
Potential side effects of surgery for vulva cancers include lymphoedema of the legs, where there is swelling of the legs after the lymph nodes of the groin are removed. It reduces over a period of time but can be persistent in some patients. Other side effects include delayed wound healing around the vulva, formation of scar tissue around the vagina leading to narrowing of the vagina.
Surgery In Advanced vulva Cancer
In advanced vulva cancer, when there is spread of cancer to nearby organs such as urinary bladder, rectum, cervix or uterus, surgery is extended to help removal of these structures. In some instances, a procedure called exenteration is done where removal of all structures in the pelvis is done to get control over the disease.
Radiotherapy
Radiotherapy is an option of treatment in vulva cancer. Radiotherapy is used as adjuvant treatment after surgery in certain patients. Who needs radiotherapy, depends on the results after surgery. These usually include patients with lymph nodes involved with cancers, patients who have tumours larger than 4cm in size or patients in whom the margins are close or contained cancer. Radiotherapy alone or radiotherapy combined with chemotherapy is an option in this setting.
Radiotherapy combined with chemotherapy is an option of treatment for patients with advanced cancer in whom surgical removal is not possible. The chemo-radiotherapy treatment is given over a period of 5-6 weeks with radiotherapy done 5 days a week and chemotherapy given once a week.
Side effects of radiotherapy include tiredness, redness and soreness of the skin around the vulva, some burning sensation when passing urine, diarrhoea and lymphoedema. Radiotherapy can cause scarring of the vaginal area leading to narrowing and using dilators after treatment can help reduce this side effect.
Chemotherapy
Chemotherapy treatment in vulva cancer is used along with Radiotherapy as part of Chemoradiotherapy. Here chemotherapy is with drugs such as Cisplatin (most common), 5- Fluorouracil and Mitomycin. When Cisplatin is used, it is given once a week for 5-6 weeks.
Chemotherapy is used on its own in patients with very advanced cancer, usually stage 4b. Here the aim of treatment is to control the disease and symptoms rather than cure. Commonly used drugs here include Carboplatin and Paclitaxel.
