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Vulvar cancerC51.-

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Carcinoma, vulva carcinoma; Carcinoma Vulva carcinoma; Vulva cancer; vulvar cancer

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DefinitionThis section has been translated automatically.

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.

ClassificationThis section has been translated automatically.

The "in situ" stage of vulvar carcinoma is referred to in extended classifications as VIN (vulvar intraepithelial neoplasia) analogous to other intraepithelial neoplasias. These include AIN (anal intraepithelial neoplasia) and CIN (cervical intraepithelial neoplasia).
Clinical stages of vulvar cancer (FIGO classification)

Stadium

Findings

I

Tumour diameter < 2 cm; limited to vulva or perineum

Ia

Stroma invasion < 1.0 mm

Ib

Stroma invasion > 1.0 mm

II

Tumour diameter > 2.0 cm; limited to vulva and perineum, no LK metastases

III

Tumour of any size with extension to the distal urethra, vagina or anus and/or LK metastases

IV

 tumour invasion into neighbouring organs, bilateral inguino-femoral LK metastases, distant metastases

Occurrence/EpidemiologyThis section has been translated automatically.

  • 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

EtiopathogenesisThis section has been translated automatically.

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:

LocalizationThis section has been translated automatically.

Mostly the labia majora are affected, more rarely the labia minora and the clitoral area.

Clinical featuresThis section has been translated automatically.

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.

DiagnosisThis section has been translated automatically.

  • 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)
Table 2: Metastatic tendency of the vulvar carcinoma
Penetration depth of the vulvar carcinoma Carcinomas with lymph node metastases
< 1.0 mm no metastasis
1.1-3.0 mm 8%
3.1-5.0 mm 27%
> 5,0 mm 34%
  • 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).

TherapyThis section has been translated automatically.

There are different forms of treatment for vulvar cancer, depending on its location, size and stage. The preferred therapy is the most complete surgical removal of the tumour by the gynaecologist. The aim is to preserve the organs and this should be carried out in specialised gynaecological centres.

ProphylaxisThis section has been translated automatically.

In a multicenter study, a quadrivalent HPV vaccine (Gardasil) was shown to significantly reduce HPV 6, 11, 16 and 18-induced infections in young women aged 16-23 years. Precancerous dysplasia or genital warts were not observed.

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer