Vulvar cancer – a relatively rare tumor. In Russia, in the structure of the incidence of malignant neoplasms of the female genital organs, vulvar cancer is from 3 to 8% and takes fourth place. In the US, vulvar cancer accounts for about 4% of all cases of cancer of the female reproductive sphere and 0.6% of all cases of cancer in women. The average age of patients with vulvar cancer at the time of detection of the disease is 68 years. In girls and women of reproductive age, vulvar cancer is very rare. With aging, especially in people over 70 years old, the likelihood of its development increases, and in people over 85 years old almost reaches the incidence of cancer of the cervix and body of the uterus. Thus, vulvar cancer is a disease of elderly women. Clinically, the most significant histological forms are malignant neoplasms developing from the integumentary squamous epithelium of the external genital organs. Squamous cell carcinoma of varying degrees of differentiation is observed in 90% of patients with malignant tumors of the vulva. Other types of vulvar tumors include malignant tumors of epithelial origin (changes in squamous epithelium – basal cell carcinoma; changes in glandular epithelium – extramillar cancer of Paget, cancer of bartholin gland, cancer from ectopic breast tissue, cancer of sweat glands, other adenocarcinomas); soft tissue malignancies (rhabdomyosarcoma, aggressive angiomyxoma, leiomyosarcoma, swelling dermatofibrosarcoma, malignant fibrous histiocytoma, epithelioid sarcoma, malignant schwannoma, malignant hemangioendothelioma, soft tissue hemorrhoids, hemoparcoma, kaposomesarcoma, hemoparcoma hemorrhoids, hemoparcoma of the liposome other malignant tumors (melanoma, hemoblastoses, yolk sac, tumor from Merkel cells, metastatic tumors). The primary source of metastatic tumors is more oftentotal is squamous cell carcinoma of the cervix, then – cancer of the endometrium, bladder and urethra. Metastases to the vulva of cancer of the vagina, mammary glands, ovaries, kidneys, skin melanoma, choriocarcinoma, lung cancer, and lymphoma are less likely.
Etiology and pathogenesis of vulvar cancer
The etiology and pathogenesis of vulvar cancer are not well understood. The development of the disease is often induced by hormonal changes occurring in the body of a woman in menopause and postmenopausal periods. The etiological classification of squamous cell carcinoma of the vulva includes at least two groups: the smaller – basaloid and warty tumors induced by human papillomaviruses (HPV), and the larger – squamous cell carcinoma, the etiology of which is unknown.
Risk factors
Risk factors: age – 3/4 patients older than 50 years, 2/3 patients older than 70 years; chronic infection with HPV, HIV; sclerosing lichen; melanoma or atypical nevi on the skin outside the vulva; low socio-economic status; intraepithelial neoplasia (VIN); genital cancer of a different location; obesity; arterial hypertension; diabetes; frequent change of sexual partners; smoking.
Metastasis of vulvar cancer
Frequent and rapid metastasis of vulvar cancer is due to a richly developed lymphatic network that covers the entire vulva and leads the lymph primarily to the inguinal, femoral (deep inguinal) and iliac lymph nodes. The outflow of lymph from the posterior parts of the vulva occurs in the inguinal lymph nodes located on the localization side of the tumor, from the anterior sections the outflow occurs in the same lymph nodes and, in addition, through the pre-symphysical plexus to the inguinal lymph nodes located on the opposite side. The outflow of lymph from the clitoris through the suprapubic and subsubic lymphatic vessels – into the intra pelvic nodes located on both sides.
The leading sign of clinical aggression of a tumor is the presence of its metastases. Clinical tumors are characterized by an extremely malignant course in cases where the phasing of lymphogenous metastasis is impaired. The frequency of metastasis of vulvar cancer to the lymph nodes of the inguinal-femoral collector in operable patients is 30-50%, at the first stage superficial inguinal lymph nodes are affected, at the second – deep, located around the main femoral vessels. Of particular clinical importance is metastasis to the proximal group of deep inguinal (femoral) lymph nodes (Cloquet-Rosenmüller-Pirogov), after which metastases appear in the iliac and obturator lymph nodes. Of the iliac lymph nodes, the external iliac lymph nodes are affected. The situation becomes fatal with metastases in the lumbar lymph nodes.
The frequency of regional metastases depends on the size of the primary tumor: with sizes up to 2 cm, metastases are found in 25–33% of cases; 2-3 cm – 60-68%; 4-7 cm – more than 90%. As the depth of tumor invasion increases, the frequency of regional metastases increases. In case of clitoral cancer, 10-30% of patients may experience isolated tumor metastases in the iliac lymph nodes with intact lymph nodes of the inguinal-femoral collector. Metastasis is cross, i.e. contralateral to tumor localization. Hematogenous metastases in vulvar cancer are observed, as a rule, in the presence of metastases in the iliac, paraaortic and paracaval lymph nodes.
Regional for vulvar cancer are the inguinal and femoral (deep inguinal) lymph nodes.
Clinical classification according to the TNM system (2003)
Primary tumor (T)
Tx – insufficient data to evaluate the primary tumor TO – primary tumor not determined Tis – cancer in situ ( pre-invasive carcinoma) T1 – the tumor is limited to the vulva and / or perineum, less than 2 cm in the largest dimension T1a – depth of the stroma invasion is less than 1 mm T1b – the depth of invasion of the stroma bolee1 mm T2 – tumor limited to the vulva and / or perineum, bolee2 cm in greatest dimension TK – tumor of any size, which covers the lower part of the urethra and / or the vagina or anal ring T4 – tumor of any size, rasprostranyayuschayas any of the following structures: mucosal upper portion of the urethra and / or bladder and / or rectum, and / or the tumor is fixed to the pelvis bones condition regional lymph nodes (N) Nx – insufficient data to evaluate the status of regional lymph nodes N0 – no metastases to regional lymph nodes N1 – metastases in regional lymph nodes with one hand N2 – metastases in regional lymph nodes on both sides of distant metastases (M) Mx – not enough data to determine a Dalen metastases Defense – no distant metastases Ml- distant metastases (including pelvic metastases in the lymph nodes) M1a – metastases in pelvic lymph nodes Mlb – other distant metastases Grouping by stages Stage 0 Tis N0 Stage IA N0 T1a Stage IB Tib N0 Stage II T2 N0 Stage III Tl, T2 N1 TK N0, N1 Stage IVA T1.T2, TK N2 T4 any N Stage IVB any T any N