oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.
This is usually a squamous cell carcinoma involving the posterior wall of upper third of the vagina. It may directly invade the bladder or rectum. Lesions may be ulcerative or exophytic.
- Those in the upper vagina metastasise in a similar way to cervical carcinoma, eg regional lymph nodes and para-aortic nodes.
- Those in the middle can invade in either direction.
- Tumours in the lower third metastasise mainly to inguinal nodes.
The distinction between squamous cell carcinoma and adenocarcinoma is important because the two types represent distinct diseases, each with a different pathogenesis and natural history:
- Approximately 85% of cases are squamous cell vaginal cancer. This initially spreads superficially within the vaginal wall and later invades the paravaginal tissues and the parametria. Distant metastases occur most commonly in the lungs and liver.
- Approximately 15% of cases are adenocarcinoma. This has a peak incidence between 17 and 21 years of age and differs from squamous cell carcinoma by an increase in pulmonary metastases and supraclavicular and pelvic node involvement.
- 80% of vaginal carcinoma is metastatic:
This may occur from:
- Bartholin's gland
Rarely, melanoma and sarcoma are described as primary vaginal cancers.
Adenosquamous carcinoma is a rare and aggressive mixed epithelial tumour comprising approximately 1-2% of cases. Clear cell adenocarcinomas plus vaginal adenosis are most often associated with in utero exposure to diethylstilbestrol.
Primary vaginal cancer can only be diagnosed if the cervix is uninvolved or only minimally involved by tumour obviously of vaginal origin. Where malignancy involves both the cervix and vagina and histology indicates either origin, then it is conventionally denoted as a cervical carcinoma.
Primary vaginal cancer is the rarest gynaecological cancer - 1-2% of all gynaecological cancers.
The National Cancer Data Base (NCDB) in the USA reported that between 1985-1994 there were 4,885 cases of primary vaginal cancer. More than 90% were epithelial neoplasia, with approximately 25% of these in situ lesions only. Squamous carcinoma was more common as the age of the patient progressed. Adenocarcinomas represented nearly all the carcinomas in the group of patients age <20 years and were observed less frequently with advanced age.
HIV-positive women have more vaginal intraepithelial lesions compared with negative controls.
In a recent study, the incidence of vulvar, vaginal or anal intraepithelial neoplasia was 1.96 per 100 person years for the HIV-infected women and 0.26 per 100 person years for the control women.
- Vaginal bleeding or bloody discharge may be seen.
- Advanced tumours may affect the rectum or bladder, or extend to the pelvic wall causing pain or leg oedema.
- Colposcopy; because vaginal intraepithelial neoplasia (VAIN) is associated with other genital neoplasias, the cervix (when present) and vulva should be examined carefully.
- Cervical cytology.
- Endometrial biopsy.
- CT scan.
- Fluorodeoxyglucose-positron emission tomography (FDG-PET) - may be more sensitive than CT scanning.
- Chest X-ray.
- Intravenous pyelogram (IVP).
- Stage 0 - squamous cell carcinoma in situ; this disease is usually multifocal and commonly occurs at the vaginal vault.
- Stage I - the disease is limited to the vaginal wall mucosa.
- Stage II - the disease involves the subvaginal tissue, but not the pelvic wall.
- Stage III - the disease extends to pelvic wall.
- Stage IV - the disease either extends beyond the true pelvis or involves the bladder or rectal mucosa.
- Stage IVA - the disease has spread to adjacent organs.
- Stage IVB - the disease has spread to distant organs.
This depends on the clinical condition of the patient and clinical staging:
- There is evidence that the majority of women have had a hysterectomy prior to the diagnosis of vaginal cancer. Most of this group develop cancers in the upper third of the vagina.
- For patients with early stage vaginal carcinoma, standard treatment is highly effective.
- For patients with stages III and IVA disease, radiation therapy alone is standard. For patients with stage IVB disease, current therapy is inadequate and there is no established standard treatment. These patients are rare and should be considered as candidates for clinical trials.
There is some evidence that 5% imiquimod cream may be useful in the treatment of vaginal intraepithelial neoplasia. Treatment leads to complete response in a large number of patients and those with only partial response will require less radical surgery. Reported side-effects were local burning and soreness, but patients did not discontinue treatment.
Combined external beam and internal radiotherapy are used.
Carbon dioxide laser is a safe and effective tool in premalignant disease. Sexual function is not compromised.
Stage I and some stage II patients may undergo radical hysterectomy with removal of the upper vagina.
There is limited evidence on the feasibility of conservative surgery in women aged under 40 years, wishing to preserve sexual and reproductive function. In one report 4 women are disease-free at 51 months' follow-up after tumour removal and pelvic lymphadenopathy with subsequent radiotherapy.
Prognosis depends primarily on the stage of disease, but survival is reduced in patients who:
- Are older than 60 years of age.
- Are symptomatic at the time of diagnosis.
- Have lesions of the middle and lower third of the vagina.
- Have adenocarcinoma rather than squamous cell carcinoma.
- Have poorly differentiated tumours.
The length of vaginal wall involvement has been found to be significantly correlated to survival and stage of disease in squamous cell carcinoma patients.
Further reading & references
- Gynaecological cancer - suspected, Clinical Knowledge Summaries (2005)
- Bardawil T et al; Surgical Treatment of Vaginal Cancer, eMedicine, Sept 2010
- Vaginal Cancer (PDQ) - National Cancer Institute (US Guidelines)
- Vaginal cancer statistics - UK, Cancer Research UK
- Gallup DG, Talledo OE, Shah KJ, et al; Invasive squamous cell carcinoma of the vagina: a 14-year study.; Obstet Gynecol. 1987 May;69(5):782-5.
- Herbst AL, Robboy SJ, Scully RE, et al; Clear-cell adenocarcinoma of the vagina and cervix in girls: analysis of 170 registry cases.; Am J Obstet Gynecol. 1974 Jul 1;119(5):713-24.
- Creasman WT, Phillips JL, Menck HR; The National Cancer Data Base report on cancer of the vagina.; Cancer. 1998 Sep 1;83(5):1033-40.
- Jamieson DJ, Paramsothy P, Cu-Uvin S, et al; Vulvar, vaginal, and perianal intraepithelial neoplasia in women with or at risk for human immunodeficiency virus.; Obstet Gynecol. 2006 May;107(5):1023-8.
- Lamoreaux WT, Grigsby PW, Dehdashti F, et al; FDG-PET evaluation of vaginal carcinoma. Int J Radiat Oncol Biol Phys. 2005 Jul 1;62(3):733-7.
- Curran WJ Jr, Whittington R, Peters AJ, et al; Vaginal recurrences of endometrial carcinoma: the prognostic value of staging by a primary vaginal carcinoma system.; Int J Radiat Oncol Biol Phys. 1988 Oct;15(4):803-8.
- Vaginal Cancer (PDQ) - National Cancer Institute (US Guidelines)
- Stock RG, Chen AS, Seski J; A 30-year experience in the management of primary carcinoma of the vagina: analysis of prognostic factors and treatment modalities. Gynecol Oncol. 1995 Jan;56(1):45-52.
- Iavazzo C, Pitsouni E, Athanasiou S, et al; Imiquimod for treatment of vulvar and vaginal intraepithelial neoplasia. Int J Gynaecol Obstet. 2008 Apr;101(1):3-10. Epub 2008 Jan 28.
- Stryker JA; Radiotherapy for vaginal carcinoma: a 23-year review.; Br J Radiol. 2000 Nov;73(875):1200-5.
- Cutillo G, Cignini P, Pizzi G, et al; Conservative treatment of reproductive and sexual function in young woman with squamous carcinoma of the vagina.; Gynecol Oncol. 2006 Apr 2;.
- Otton GR, Nicklin JL, Dickie GJ, et al; Early-stage vaginal carcinoma--an analysis of 70 patients. Int J Gynecol Cancer. 2004 Mar-Apr;14(2):304-10.
- Eddy GL, Marks RD Jr, Miller MC 3rd, et al; Primary invasive vaginal carcinoma.; Am J Obstet Gynecol. 1991 Aug;165(2):292-6; discussion 296-8.
- Dixit S, Singhal S, Baboo HA; Squamous cell carcinoma of the vagina: a review of 70 cases.; Gynecol Oncol. 1993 Jan;48(1):80-7.
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Dr Hayley Willacy
Dr Hayley Willacy