Vulvar cancer is a malignant tumor lesion of the external genital area (labia, clitoris, vaginal vestibule, posterior adhesions, urethra, etc.). Manifestations can be itching, irritation, discomfort in the genital area, pain, ulceration, exophytic formations, bloody or purulent and discharge. Diagnosis includes gynecological examination, vulvoscopy, cytological examination of smears and histological analysis of biopsy material. Surgical, chemotherapeutic and radiation methods are used in the treatment of vulvar cancer.
In the structure of malignant oncogynecological pathology, disease is about 2-5%, inferior in frequency to cervical cancer, endometrial cancer and ovarian cancer. More often, vulvar cancer develops in women 65-75 years old; it is rarely diagnosed at reproductive age. The typical localization is the labia, clitoris, urethra, posterior spike, less often the process affects the area of the bartholinium glands.
Despite the fact that vulvar cancer refers to tumor processes of external localization, more than 60% of patients turn to a gynecologist already at stage III–IV of the disease. This problem in gynecology is evidence of low medical culture, insufficient preventive work, untimely detection of background and precancerous lesions of the vulva.
The issues of epidemiology are not sufficiently disclosed. It is believed that the mechanisms leading to the development of vulvar cancer are triggered as a result of hormonal restructuring of the reproductive sphere during menopause and postmenopause. This leads to a decrease in the level of sensitivity of receptors to estrogen, age-related involutive changes in tissues. Under unfavorable conditions, proliferative, dysplastic changes occur in the tissues of the vulva, followed by malignant transformation.
Disease often develops against the background of previous dystrophic processes of the genitals – kraurosis and leukoplakia of the vulva, sclerosing lichen. The true precancer in gynecology includes vulvar dysplasia (atypical hyperplasia). Among the factors that significantly increase the likelihood are the presence of papillomavirus infection, HIV, melanoma or atypical nevi, obesity, arterial hypertension, diabetes mellitus, late onset of menarche and early menopause. A significant role in the development of vulvar cancer is played by bad habits, frequent change of sexual partners, STIs.
According to the histotype, in 80-90% of cases, a squamous cell form with a high degree of differentiation is diagnosed. In 2% of patients, malignant melanoma of the vulva is detected; much less often – other forms (primary adenocarcinoma, Paget’s cancer, basal cell carcinoma, sarcoma, cancer of the bartholin gland). According to the type of growth, there are exophytic, nodular, ulcerative, infiltrative-edematous cancer of the vulva.
According to the international clinical classification, the prevalence of vulvar cancer is assessed by FIGO stages (0-IV) and TNM criteria (where, T is the primary tumor, N is regional lymph nodes, M is distant metastasis):
Stage 0 – (Tis) – preinvasive vulvar cancer.
Stage I – (T1) – the spread of the tumor is limited to the vulva or perineum, but does not exceed 2 cm in the largest size:
- IA (T1A) – invasion of stroma less than 1 cm
- IV (T1B) – invasion of the stroma more than 1 cm
Stage II (T2) – the spread of the tumor is limited to the vulva or perineum, but exceeds 2 cm in the largest size.
Stage III (T3 N1) – tumor growth can spread to the vagina, lower urethra, anus, regional lymph nodes on one side.
Stage IVA (T4 N2) – tumor growth can spread to the bladder mucosa, upper urethra, rectum, lymph nodes on both sides.
Stage IVB (M1) – with any prevalence of vulvar cancer, distant metastases are determined.
Symptoms of vulvar cancer
Complaints of vulvar cancer can be diverse. Typical early symptoms are burning, irritation, itching of the vulva, having a paroxysmal character and intensifying at night. As the tumor increases and tissue trophic disorders, ulceration of the mucosa, wetness appears. Edema and violation of the integrity of the vulva cover are manifested by pain, serous, bloody or purulent (with secondary infection) secretions. The pain syndrome is especially pronounced with a tumor of the clitoris and deep infiltration of tissues. When the tumor disintegrates, massive bleeding and abundant fetid discharge may occur. With advanced vulvar cancer, there is swelling of the pubis, extremities, violation of urination, constipation.
Metastasis of vulvar cancer occurs in a lymphogenic way; at the same time, the inguinal, then the iliac and subcutaneous lymph nodes are initially affected. Common manifestations of vulvar cancer in the late stages are subfebrility, fatigue, weakness, and decreased ability to work. Vulvar cancer is differentiated with vulvitis, bartholinitis, syphilis, tuberculosis, benign neoplasms of the vulva, skin diseases (dermatomycoses, dermatoses, pigmented formations).
The primary method of recognizing vulvar cancer is a gynecological examination. Visually, in the area of the labia minora or labia majora, clitoris, urethra, small nodules, ulceration, warty or pigmented formations may be detected, differing in color from unchanged tissues. For a detailed examination of atypical areas of the vulva, vulvoscopy (colposcopy) is performed.
In the process of diagnosis, taking and examination (microscopic, cytological, bacteriological) of smears from the surface of the neoplasm, from the vagina and cervical canal is performed. A decisive method in the diagnosis is a biopsy of the external genitalia, followed by a histological examination of the material.
To stage vulvar cancer, determine the degree of the process and the presence of metastases, ultrasound of the pelvis, ultrasound of the abdominal cavity and lymph nodes, chest X-ray; according to indications – cystoscopy, anoscopy, rectoscopy. If necessary, the patient is consulted by a pulmonologist, proctologist, urologist.
Surgical and radiation treatments are most widely used for vulvar cancer; photodynamic therapy, laser ablation and chemotherapy are also possible. At stage 0 (vulvar cancer in situ), a wide excision (excision) of the tumor focus or ablation of the vulva is performed. With a more common lesion and invasive growth (stage I), a vulvectomy with unilateral lymphadenectomy is indicated. Vulvectomy consists in removing the clitoris, labia, and fatty tissue in the pubic area.
Detection of vulvar cancer in stage II is the basis for radical vulvectomy with bilateral inguinal-femoral lymphadenectomy. If the tumor is localized in the clitoris, femoral and inguinal-iliac lymphadenectomy is required. If the lymph nodes are interested, the surgical stage of removal of vulvar cancer is supplemented by postoperative irradiation of regional zones. With resectable vulvar cancer at stage III, treatment begins with radical vulvectomy, bilateral inguinal-femoral-iliac lymphadenectomy followed by remote irradiation of all interested areas.
The possibilities of surgical treatment at stage IV depend on the prevalence of the tumor lesion on neighboring structures. In some cases, radical vulvectomy, removal of the rectum, bladder, vagina, fiber, removal of the uterus (hysterectomy) with adnexectomy and further irradiation of metastasis zones are performed. In case of unresectable vulvar cancer, treatment begins with radiation (chemoradiotherapy) therapy, then, if possible, a surgical stage is performed.
The subsequent prognosis is determined by the initial stage of vulvar cancer and the radicality of the treatment. The most aggressive course has cancer of the vulva, localized in the clitoris, as well as with an infiltrative-edematous type of growth; the most favorable – tumors of the labia minora; intermediate – cancer of the labia majora.
The three-year survival rate with combined therapy of vulvar cancer is 63-70%, five-year – 57-60%; with radiation therapy alone – 50% and 30%, respectively.