Definition and epidemiology
Cancer of the vulva is mostly a disease of older (60 – 70 years) women.
The prognosis of vulvar cancer is poor, even though most are discovered in an early stage.
Squamous cell carcinoma is the most common histological type, accounting for 90% of cases. The second most common is adenocarcinoma, which originates from the Bartholin glands.
- HPV infection
- Vulvar dermatosis (like lichen sclerosus)
Vulvar intraepithelial neoplasia
Vulvar intraepithelial neoplasia (VIN) is the premalignant precursor to vulvar cancer. These lesions are often multifocal (many foci within the same organ) and multicentric (concomitant foci in other organs).
VIN is classified according to the ISSBD 2015 system. According to this system, the cells can either be:
- Low-grade squamous intraepithelial lesion (LSIL)
- High-grade squamous intraepithelial lesion (HSIL)
- Differentiated vulvar intraepithelial neoplasia (dVIN)
LSIL is a benign lesion and is not premalignant. HSIL and dVIN however, are associated with the development of vulvar cancer.
Vaginal intraepithelial neoplasia
Vaginal intraepithelial neoplasia (VAIN) is the premalignant precursor to vaginal cancer.
VAIN is classified similarly to VIN:
- Low-grade squamous intraepithelial lesion (LSIL) – also called VAIN 1
- High-grade squamous intraepithelial lesion (HSIL) – also called VAIN 3
VAIN 2 is an intermediate lesion between LSIL and HSIL which is sometimes used, but it is usually reclassified as LSIL or HSIL upon closer pathological review.
Symptoms and diagnosis of VAIN is similar to that of CIN. It can be screened for by Pap smear, it can be diagnosed by biopsy. It is treated surgically.
|I||Tumor confined to the vulva and/or perineum|
|II||Tumor of any size with extension to adjacent perineal structures (distal third of the urethra, distal third of the vagina, anal involvement)|
|III||Tumour of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguinal-femoral lymph nodes|
|IV||Tumour invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures|
(Substages excluded for simplicity)
Patients with VIN or vulvar cancer usually present with a vulvar lesion, which is visible but often asymptomatic. If symptomatic, it can be itchy or bleeding.
Diagnosis and evaluation
Diagnosis is made based of biopsy of the lesion. It can be difficult to distinguish VIN from lichen sclerosus or lichen planus, so biopsy should be taken of all lesions of the vulva which are not certain to be benign.
LSIL is not premalignant and does not require treatment unless symptomatic. VIN can be treated by surgical excision or by ablation. Unfortunately, recurrence of VIN is common, so patients should be monitored regularly for recurrence.
The treatment of vulvar cancer is generally surgical. The radicality of the surgery depends on the stage, the age of the patient, the comorbidities, and the stages of the lymph nodes. The options are:
- Local excision with 1 cm free margin or partial vulvectomy are options in case of young age + small tumour.
- Radical vulvectomy ± inguinofemoral lymphadenectomy ± pelvic lymphadenectomy
- Pelvic exenteration is the most radical treatment option. It involves removing all organs from the pelvic cavity.
The stages of the lymph nodes should be determined by sentinel lymph node biopsy. The sentinel lymph nodes in case of vulvar cancer are called Cloquet or Rosenmüller lymph nodes. If the sentinel lymph node is positive, regional lymphadenectomy is necessary.
Chemotherapy may be an option in recurring or high stage cancers.
In case of palliative cases, electro-excision or laser-excision and coagulation can be used to resect tumours in which surgical stitching is impossible.
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