Cancer of the vulva

Cancer of the vulva

The vulva
The vulva is a collective term used to refer to the external sex organs of a woman as well as the skin that surrounds them. The vulva is made up of:
•a•? The opening of the vagina
•a•? Two labia minora and labia majora
•a•? The opening of the urethra
•a•? The clitoris

Cancer of the vulva
This cancer is the most common carcinoma of the labia majora, labia minora or clitoris; it can originate as ureteral tumour. Cancer of the vulva is amongst the top five gynaecological cancers. In three out of four women the cancer first develops in one of the woman’s labia. The global incidence of this disease is not precisely known: however, the incidence of pre?invasive disease of the vulva has nearly doubled over the last ten years and potentially this could in the future translate into a marked increase in the incidence of invasive vulvar carcinoma.
Vulvar carcinoma incidence has a bimodal peak. Development of vulvar carcinoma in situ in young women may correlate to human papilloma virus (HPV) infection. In older women, the cause of the carcinoma is attributed to chronic irritation or other poorly understood cofactors. Women who smoke cigarettes have a 4–5?fold increase in the incidence of carcinoma in situ of the vulva and a 20% increase in vulvar carcinoma. The incidence is higher in those women who have multiple sexual partners and those with a history of HPV infection.

Pathophysiology
Vulvar cancer is rare and accounts for less than 1% of all cancer cases in the UK. It is extremely rare in women aged under 25; however, rates increase to 3.8 per 100,000 in women aged 60–64. It is highest in those aged 85 and over. The majority of vulvar cancers (98%) are due to squamous cell carcinoma. The rest are made up of basal cell carcinomas, adenocarcinomas, sarcomas and melanomas. The labium majorum is the most common site of involvement, accounting for about 50% of cases. The labium minorum accounts for approximately 20% of cases. The clitoris and Bartholin’s glands are not as commonly involved. The cancer often spreads slowly, locally and metastasizes to groin nodes and then from there to A pelvic nodes.
The development of vulvar dysplasia and cancer in most women is related to HPV infection. HPV types 16, 18, 31, 33, 35, 45 and 54 are known to be more oncogenic than others. They are more likely to be associated with cervical neoplasia and cancer; these are likely to be responsible for vulvar cancers. The mechanism of HPV transformation into dysplasia and cancer is poorly understood.

Signs and symptoms
Often the diagnosis of vulvar carcinoma is delayed. It is usual for some woman to fail to seek treatment for six months on average from the onset of symptoms. In addition, there may also be a delay in diagnosis after the patient presents to the clinician. In a number of cases, a biopsy of the lesion is not performed until the problem fails to respond to a variety of topical therapies. Biopsy should be performed when any distinct lesion of the vulva is noticed. For most women the most common presentation is a pruritic lesion of the vulva or a mass that has been detected by the patient herself. There may be bleeding due to ulceration and pain. Early vulvar cancer, however, may be asymptomatic and noticed only with careful inspection of the vulva. All visible lesions on the vulva should be biopsied. Bleeding, pain or discharge are associated with more advanced vulvar carcinomas.
The pattern of spread is associated with the histology. Well?A differentiated lesions often spread along the surface, with minimal invasion; anaplastic lesions, however, are more likely to be deeply invasive. Spread beyond the vulva is due to either adjacent organs for example the vagina, urethra and anus, or via the lymphatics to the inguinal and femoral lymph nodes, followed by the deep pelvic nodes. About 5% of cases will spread to distant sites.

Investigations
The diagnosis of vulvar cancer is made by biopsy. For staging A purposes other investigations are carried out, including cystoscopy, proctoscopy, chest X?ray and intravenous urography. A CT scan may be useful to help evaluate nodal spread in the pelvis if there is evidence of groin node metastasis. There may also be a need to perform laparoscopic assessment of the pelvic lymph nodes as an alternative to CT scan. Colposcopy may be performed on the vulva; however, this is more difficult than colposcopy of the cervix due to the large A surface area of the vulva and the variability in premalignant lesions.
A punch biopsy can be used to take a representative sample of the vulva. The need for biopsy has already been made. To reiterate, biopsy should be performed on all lesions to ensure that a cancer is not missed when multiple dysplastic lesions are present.

Staging
It is important to stage the cancer, informing the clinician how large the cancer is and how far it might have spread. The various investigations and tests also provide some information about the stage; treatment is usually decided according to the stage of a cancer.
In the UK, vulvar cancer is often staged according to the A definitions by the Federation Internationale de Gynecologie et d’Obstetrique (FIGO). There are four main stages in this system

Management
The main treatment for vulvar cancer is surgery and the type of surgery depends on the site and extent of the primary lesion and the risk of lymph node involvement. The aim of surgery is to remove cancerous tissue from the vulva, while also trying to minimize the impact of surgery on the vulva. However, in some instances this may not be possible. A combination of radiotherapy and chemotherapy may also be used as the main treatment if surgery would lead to urinary incontinence or faecal incontinence, or if the woman requests this. This approach can also be used to reduce the spread of advanced cancer if curative approaches are not possible (palliative care).










Menorrhagia
Menorrhagia refers to menstrual blood loss that interferes with a woman’s physical, emotional, social and material quality of life; this can occur alone or in combination with other symptoms. The aim of any intervention should be to improve a woman’s quality of life. A monthly menstrual blood loss in excess of 80a•›ml is said to be menorrhagia.

Menorrhagia
The average menstrual cycle has a blood loss for 7 days of a cycle of between 21 and 35 days (Ka•›=a•›7/21–35, where K represents menstrual cycle). For the first few days menstrual loss is heaviest, then it becomes much lighter and tails off towards the end. Other definitions include:
•a•? Metrorrhagia – menstrual flow at irregular intervals
•a•? Menometrorrhagia – flow that is frequent and excessive
•a•? Polymenorrhoea – bleeding at intervals less than 21 days
•a•? Dysfunctional uterine bleeding – abnormal uterine bleeding with no obvious structural or systemic pathology
•a•? Dysmenorrhoea – the experience of pain with menstruation
The normal menstrual blood loss is approximately 35–40a•›ml.
As menorrhagia is very subjective, a more practical definition may be that it is menstrual loss that is greater than the woman feels she can reasonably manage. Heavy menstrual loss is excessive blood loss that interferes with a woman’s physical, social, emotional and/ or quality of life.

Pathophysiology
Approximately half of those women who complain of excessive bleeding have no pathology; this is known as dysfunctional uterine bleeding. Approximately 20% of cases are associated with anovulatory cycles and these are most common at the extremes of reproductive life. Local causes can include:
•a•? Fibroids
•a•? Endometrial polyps
•a•? Adenomyosis
•a•? Endometritis
•a•? Endometrial hyperplasia
•a•? Pelvic inflammatory disease
•a•? Endometrial carcinoma
Systemic disease can include hypothyroidism, liver or kidney failure and bleeding disorders. An IUD can increase A menstrual flow.

Signs and symptoms
Symptoms related by the woman with menorrhagia may often be more instructive than laboratory tests. It is essential to undertake a detailed patient history. Figure 47.1 outlines some essential issues that should be addressed in the history?taking phase. The physical examination should be modified in order to meet the needs of the woman. The clinician should observe for signs of severe anaemia, this may confirm the patient’s history of very heavy bleeding and prompt immediate in?patient care. Obesity is an independent risk factor for endometrial cancer. Adipose tissue is ideal for oestrogen conversion. Signs of androgen excess (hirsutism) may be PCOS, leading to anovulatory bleeding. Ecchymosis is usually a sign of trauma or a bleeding disorder; purpura is also a sign of trauma or a possible bleeding disorder. Uterine size, shape and contour should be assessed. Adnexal tenderness or masses could indicate ovarian cancer; intermenstrual bleeding may be its only symptom. Finding an adnexal mass should prompt an immediate pelvic ultrasound.

Investigations
A full blood count is required (menorrhagaia is the most common cause of iron deficiency anaemia in women). Tests for endocrine irregularities, including thyroid function tests, may be needed. If there is a clinical suspicion, then undertake an assessment of A bleeding disorders. Biopsy should be carried out in order to exclude endometrial cancer or atypical hyperplasia. A trans?A vaginal ultrasound is the first?line diagnostic tool for identifying structural abnormalities such as fibroids. Cervical specimens should be taken if the woman is at risk of an infection.

Management
Pharmaceutical treatment is the preferred treatment; when firstline pharmacology treatment is ineffective then a second pharmaceutical treatment should be considered as opposed to an immediate referral to surgery. Medical therapy for menorrhagia should be tailored to the individual. Factors taken into consideration when selecting the appropriate medical treatment include the patient’s age, coexisting medical diseases, family history and desire for fertility. The woman must be at the centre of all decisions being made.

Pharmacology
Iron deficiency should be corrected with oral iron. The levonorgestrel intrauterine system is first?line treatment, it is a long?term treatment, left in situ for at least 12 months.
Consider giving tranexamic acid, mefanamic acid or the combined oral contraceptive pill if the intrauterine system is unacceptable to the woman. A 3–4 month course of a gonadotrophin? releasing hormone analogue should be considered prior to hysterectomy or myomectomy. There is no consensus about which regimens are the most effective.

Surgical options
The type of surgical intervention will depend on uterine size and the woman’s desire to retain her uterus. Endometrial ablation is the recommended first?line surgical treatment. This involves removing the full thickness of the endometrium, together with the superficial myometrium; it retains the uterus, but is contraindicated in women with large fibroids or suspected malignancy and in those who have not completed their family. Uterine artery embolization or hysteroscopic myomectomy can be offered if the woman wishes to retain her uterus. If the patient does not wish to retain her uterus, then vaginal hysterectomy is the first considerationa, then abdominal hysterectomy with conservation of ovaries, if appropriate.