Vulvar cancer represents 3% to 5% of all female genital cancers and 1% of all malignancies in women. In 2017, there are 6,020 new cases and 1,150 deaths predicted to occur.
The average age at diagnosis is 65 years, although it is trending toward a younger age.
Clinical features include pruritus, ulceration, or a mass. The most common location of lesions is the labia majora (40%), the labia minora (20%), peri clitoral region (10%), and perineal area (15%). The route of spread is either by direct extension, lymphatic embolization to the groin nodes, or lymphatic or hematogenous spread to distant sites.
Risk factors are multifactorial: age greater than 70 years, lower socioeconomic status, hypertension, diabetes, prior lower genital tract dysplasia or cancer, immunosuppression, and human papillomavirus (HPV) infection are known to increase the risk of vulvar cancer. Vulvar SIL/dysplasia is the precancerous state and 76% of patients with vulvar HSIL are HPV positive. There is a 22% rate of subclinical invasive disease in vulvar HSIL, usually less than 1 mm DOI (31)
HISTOLOGY
Squamous cell carcinoma represents 85% of all vulvar cancers. Other histologic types are basal cell carcinoma, adenocarcinoma, sarcoma, and verrucous carcinoma and melanoma.
Malignant melanoma represents 5% of vulvar cancers. There are four histologic subtypes of melanoma: superficial spreading, lentigo, acral, and nodular.
Vulvar Paget’s disease has cutaneous and noncutaneous (bladder/colorectal) subtypes. Underlying invasive adenocarcinoma is present in 4% to 17% of cases; 30% to 42% of patients may have, or will later develop, an adenocarcinoma at another non vulvar location such as the breast, rectum, colon, or uterus.
PRE-TREATMENT WORKUP
Pre-treatment workup includes a physical exam with careful evaluation of the vagina and cervix. Five percent of invasive lesions are multifocal. Biopsy for diagnosis should occur at the center of any suspicious area.
Imaging with CT,MRI,o rPET can be obtained if positive groin or pelvic LNsare suspected. Chest x-ray should be obtained, as well as standard lab tests. EUA with cystoscopy can assist in determination of the extent of an anterior lesion’s involve ment of the urethra. Proctoscopy can be helpful in determination of anorectal involvement if there is a large lesion impinging on the posterior perineal triangle.
Vulvar cancer is a rare type of cancer that occurs in the tissues of the vulva, which is the external genital area of women. The exact causes of vulvar cancer are not fully understood, but there are several known risk factors. Here are the causes and symptoms of vulvar cancer:
Causes:
- Human papillomavirus (HPV) infection: Certain strains of HPV, particularly HPV types 16 and 18, have been linked to an increased risk of vulvar cancer.
- Age: The risk of vulvar cancer increases with age, with most cases occurring in women over the age of 50.
- Smoking: Cigarette smoking has been associated with a higher risk of vulvar cancer.
- Vulvar intraepithelial neoplasia (VIN): VIN is a precancerous condition that can progress to vulvar cancer. It is often associated with HPV infection.
- Immunodeficiency: Having a weakened immune system, such as due to HIV/AIDS or immunosuppressive medications, can increase the risk of developing vulvar cancer.
Symptoms:
- Persistent itching: Pruritus or itching in the vulvar area that does not go away or worsens over time is a common early symptom of vulvar cancer.
- Pain or tenderness: Persistent pain, soreness, or tenderness in the vulvar area may indicate vulvar cancer, especially if it doesn’t have an obvious cause.
- Changes in the skin: Any changes in the color, texture, or thickness of the skin on the vulva, such as the development of a lump, ulceration, or a wart-like growth, should be evaluated by a healthcare professional.
- Bleeding or discharge: Unexplained bleeding that is not related to menstruation or postmenopausal bleeding, as well as abnormal vaginal discharge, may be signs of vulvar cancer.
- Enlarged lymph nodes: In some cases, vulvar cancer may cause the lymph nodes in the groin area to become enlarged.
It’s important to note that these symptoms can also be caused by other non-cancerous conditions. However, if you experience any persistent symptoms or notice any unusual changes in the vulvar area, it is important to consult a healthcare professional for proper evaluation and diagnosis.
Regular pelvic exams and self-examinations of the vulva can help with the early detection of any abnormalities or signs of vulvar cancer.
TREATMENT
Management of squamous cell and adenocarcinomas has been wide radical excision (radical hemi/vulvectomy) with a 1-cm to 2-cm gross margin with groin (S)LND. For lesions that invade less than a depth of 1 mm, the groin LND can be omitted. If the lesion is lateral (more than 2 cm from the midline), dissection of the contralateral groin can be omitted. If the lesion is midline, or within 2 cm of a midline structure, a bilateral groin SLND should be performed
FOLLOW-UP
Every 3 months for the first 2 years
Every 6 months for the next 3 years
Annual visits thereafter
REFERENCES
- Iversen T, Aalders JG, Christensen A, et al. Squamous cell carcinoma of the vulva: a review of 424 patients, 1956-1974. Gynecol Oncol. 1980;9(3):271-279.
- Gonzalez Bosquet J, Kinney WK, Russell AH, et al. Risk of occult inguinofemoral lymph node metastasis from squamous carcinoma of the vulva. Int J Radiat Oncol Biol Phys. 2003;57(2):419-424.
- Klapdor R, Länger F, Gratz KF. SPECT/CT for SLN dissection in vulvar cancer: improved SLN detection and dissection by preoperative three-dimensional anatomical localisation. Gynecol Oncol. September 2015;138(3):590-596.
