Vulvar cancer C51.-

Squamous cell carcinoma in the area of the large and small labia. Squamous cell carcinoma comprises 95% of the vulvar tumours. They often develop on pre-existing lichen sclerosus et atrophicus, condylomata acuminata or at the base of chronic vulvitis. S.a. VIN (= vulvar intraepithelial neoplasia). The other malignancies of the vulva are basal cell carcinomas, adenocarcinomas, melanomas, carcinomas of the Bartholin glands.

• The proportion of tumours of the female genital tract is 3-5%.
• Incidence: 1.5/100,000 women/year; increasing with age from 0.4/100,000 women/year in 30-year-old women to 20/100,000 women/year in women over 70.
• Frequency peak of VIN exists for women in the 3rd-4th decade

Basically, a distinction can be made:

• HPV-associated vulva carcinomas
• Non-HPV-associated vulvar carcinomas

In HPV-associated vulvar carcinomas, infections with the high-risk types HPV 16, 18, 31 or 33 are responsible in most cases. Further risk factors are:

• anal carcinoma or condyloma acuminata in the medical history
• Immunosuppression
• lichen sclerosus et atrophicans
• Chronic vulvitis
• HIV infection
• Nicotine abuse.

Primarily, slow-growing, mostly sharply defined, irregularly confugured, verrucous plaques appear. Larger tumors (> 0.5 cm) are characterized by roughly indurated, irregularly configured, usually ulcerous decaying plaques or nodules that bleed easily when mechanically irritated. Vulvar carcinomas tend to metastasize early to the regional lymph nodes.

• Pretherapeutic diagnostics:
• Inspection of the vulva for changes in colour and surface relief
• Vulvoscopy after exposure to 3% acetic acid to determine representative biopsy areas
• Toluidine blue sample (Collins test): 1% toluidine blue solution, then 2% acetic acid
• Cytological smear and staining according to Papanicolaou
• Punch biopsy or excision biopsy (in case of circumscripts, on VIN suspicious foci)
• Lymph node sonography or scintigraphy
• Lymph node biopsy (see table 2)

• Pretherapeutic staging for vulvar cancer:

Staging is carried out in accordance with FIGO recommendations (see Table 1). The surgical findings and the result of the histopathological examination of the surgical specimens and the lymph nodes are decisive (see Table 2).

• Stage I-IV:
• Gynaecological examination:
• Inspection: Entire vulva, urethra, introitus, vagina, portio, perineum, anus
• Palpation: Entire vulva, vagina, internal genitals, anus, rectum, pelvic wall, groin including the thigh pits Documentation of all changes
• Imaging procedures (including vaginal sonography, lymph node sonography).
• Stage I and II:
• Useful in individual cases
• X-ray Thorax
• Liver sonography.
• Stage III:
• obligatory:
• X-ray chest
• Liver sonography
• Cystoscopy
• Rectoscopy
• useful in individual cases:
• vaginal sonography
• Rectal sonography
• Further imaging or endoscopic procedures only in case of specific indication.
• Stage IV:
• Individually adapted to the clinical situation (therapeutic consequence).

1. Expert group of the Association for Gynaecological Oncology (AGO) (2002) Vulva carcinoma. In: Brief Interdisciplinary Guidelines 2002, 3rd edition
2. Cliby W et al (1991) Stage I small cell carcinoma of the vulva treated with vulvectomy, lymphadenectomy and adjuvant chemotherapy. Cancer 67: 2415-2417
3. Conley LJ et al (2002) HIV-1 infection and risk of vulvovaginal and perianal condylomata acuminata and intraepithelial neoplasia: a prospective cohort study. Lancet 359: 108-113
4. Creasman WT (1995) New gynecologic cancer screening. Gynecol Oncol 58: 157-164
5. Hopkins MP et al (1990) The surgical management of recurrent squamous cell carcinoma of the vulva. Obstetric Gynecol 75: 1001-1005
6. Mullerat J et al (2003) Angiogenesis in anal warts, anal intraepithelial neoplasia and anal squamous cell carcinoma. Colorectal Dis 5: 353-357
7. Perez CA et al (1993) Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71: 3707-3716
8. Rodolakis A et al (2003) Vulvar intraepithelial neoplasia (VIN)--diagnostic and therapeutic challenges. Eur J Gynaecol Oncol 24: 317-322
9. Ueda Y et al (2011) Two distinct pathways to development of squamous cell carcinoma of the vulva. J Skin Cancer. 2011:951250
10. Villa LL et al (2006) High sustained efficacy of a prophylactic quadrivalent human papillomavirus types 6/11/16/18 L1 virus-like particle vaccine through 5 years of follow-up. Br J Cancer 95: 1459-1466
11. Yalcin OT et al (2003) Vaginal intraepithelial neoplasia: treatment by carbon dioxide laser and risk factors for failure. Eur J Obstet Gynecol Reprod Biol 106: 64-68