Background
It is strictly defined as
○ a cancer found in the vagina
○ without clinical or histologic evidence of cervical or vulvar cancer,
○ or a prior history of these cancers within five years
- Rare and accounts for only 1-2% of all gynaecological malignancies.
Diagnosis of a primary vaginal cancer is rare because most of these lesions will be metastatic from another
primary site - 80% of cases are metastatic or secondary tumors
Although cancer of the vagina is more common in postmenopausal women, an increase in young women being
diagnosed with primary vaginal cancer has been reported, especially in countries with a high HIV prevalence – Reason — Causes high HPV prevelance.
- Wider age range (18-95 years old) with peak incidence in the sixth decade
- Mean age approximately 60-65 years.
Aetiology
- Cause unknown
Several predisposing and associated factors
• Previous lower genital tract intraepithelial neoplasia and neoplasia
•HPV infection (oncogenic subtypes)
• Previous gynaecological malignancy
No association with the following
- Pelvic radiotherapy
- Previous hysterectomy
- Long‐term use of a vaginal pessary
•Chronic uterovaginal prolapse - Parity
Pathology
- 80-90% are squamous cell carcinomas
Others
▪ Adenocarcinoma (8-10%)
▪ adenosquamous
▪ clear cell adenocarcinomas
•
Rare types
▪ sarcomas
▪ rhabdomyosarcoma
▪ clear cell adenocarcinoma
▪ melanoma
▪ endodermal sinus tumour
•
Prevention
Primary Prevention : Vaccination
As with Cervical Cancer persistent HPV infection—particularly the HPV 16 subtype—has been associated with the
long-term development of high-grade squamous intraepithelial lesion (HSIL) and carcinoma of the vagina
- Therefore HPV vaccination is primary prevention
Secondary Prevention : Screening
- If a hysterectomy done for benign disease – No need of vault smears
If a hysterectomy has been performed for persistent HSIL after repeated excisional procedures of the cervix,
vault smears are recommended for long-term follow-up.
• - Smear + HPV testing increases accuracy and has been advised to do together
Tertiary prevention : Management of precancerous lesions
As the majority of vaginal cancers are of squamous histology, a common etiology is shared with cervical cancer.
This is the persistence of high-risk/ oncogenic HPV infections
•
- Co-factors include immunosuppression and cigarette smoking
These precancerous lesions are named Vaginal Intraepithelial Neoplasia (VAIN). Two types
LSIL – may be associated with either low-risk or high-risk HPV and it represents productive or transient
infections that may regress (previously VAIN 1).
○
HSIL – represents transforming high-risk infections (previously VAIN 2–3).
Women with HSIL are usually asymptomatic and the majority of women are aged over 60 years. HSIL
can be seen in younger women, especially in immunocompromised individuals (HIV and transplant
patients)
▪
▪ Risk of progression of HSIL to invasive cancer has been found to range between 2% and 12%.
Colposcopy with acetic acid and/or Lugol iodine is indicated if a woman has an abnormal vaginal cytological smear and no gross abnormality.
Biopsy proven LSIL lesions can be followed up with observation only (repeat smears and colposcopy), especially if women have non-oncogenic strains of HPV.
The various modalities of treatment for HSIL lesions include
a. laser ablation,
b. surgical excision,
c. topical treatments such as imiquimod
d. topical chemotherapy with 5-fluorouracil
•
Type of treatment depends on
a. number and location of the lesions,
b. the degree of suspicion for an invasive cancer
c. availability of various treatment options
d. cost of treatment
e. skill of the treating doctor
Since recurrence rates are high, it is recommended to go for more than one treatment modality
Presentation
Depends on the stage of the tumour
Vaginal bleeding (>50% of cases)
Vaginal discharge ( Odorous)
Urinary symptoms
Abdominal mass or pain
Asymptomatic (approximately 10% of tumours)
Examination
Careful examination of the vaginal walls while withdrawing the speculum is necessary to overlooking vaginal
tumours.
•
- Small ulcer (<1cm in diameter) exophytic mass large pelvic mass
Site and size
- Can occur at any site of the vagina
The upper third of the vagina is the site most frequently involved – Mainly posterior wall
▪ Either alone or together with the middle third in approximately two third of the cases
▪ 1 in 6 will be found to involve the entire length of the vagina.
•
Size
▪ varies
▪ majority of tumour are a maximum of 2-4 cm in diameter
Staging
Vaginal carcinoma is primarily clinically staged. This is based on the results of a physical exam, biopsy, and imaging
tests performed before treatment selection.
By definition, tumors in the vagina that touch or extend to the external os of the cervix should be classified as cervical cancer
Problems with current FIGO staging
- It may be difficult to differentiate one stage from another particularly between stage I and II
- It is difficult to separate stage IIa and IIb on purely clinical grounds
Differences also exists in interpretations of the significance of positive inguinal nodes and their effect on staging.
•
Assessment
Examination under anaesthesia – Is best performed under general anaesthesia
Components of EUA - The site and the limits of the tumor can be accurately determined.
- A full thickness biopsy is taken for histological analysis
Combined rectovaginal examination is helpful to determine whether there is any extension of the tumour
beyond the vagina.
•
Cystoscopy and sigmoidoscopy are required to exclude or confirm the involvement of bladder or rectum.
Radiological adjuncts
Computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET) are
encouraged to guide management; however, these tests should not be used to change the initial clinical
staging
Imaging should be used to better define tumor volume and extension of disease
- CXR and CT
- MRI – MRI is more sensitive in detecting tumor size, as well as paravaginal or parametrial involvement
PET-CT : Superior compared with other imaging modalities for detecting nodal disease. It is also useful in
detecting recurrent disease
3. - Intravenous urogram
- Rectal USS
Treatment
Depends primarily on - histology,
- tumor volume,
- anatomical localization of the lesion,
- stage of the disease,
- and age of the patient
Radiotherapy
- Is certainly effective in treating vaginal cancers
- Survival rates have improved.
- The principal advantage of radiation is organ preservation
Techniques utilized
External beam radiotherapy (teletherapy) –
EBRT to the pelvis includes the external iliac and obturator nodes as per standard of care. In addition, the
inguinal nodes may be included if the tumor is in the distal vagina
○
○ The optimal or lower threshold dose is 70 Gy
Intensity modulated radiation therapy (IMRT) is an advanced form of radiation that allows for higher
dosages of radiation to be delivered to the cancer
○
•
Brachytherapy
○ interstitial implants,
○ intravaginal cylinders or
○ vaginal ovoids.
• - A combination of the two
Majority of tumours are treated with combination of EBRT and brachytherapy.
Small early stage tumours are suitable for treatment with brachytherapy alone.
Complications of radiotherapy - Vary according to dosage and techniques used
- Occur in 12-20% of patients
- In 6%, life threatening complications have been reported.
Acute complications:
Proctitis
Radiation cystitis
Vulvar excoriation or ulceration
Vaginal necrosis
Long term complications
Vesicovaginal or rectovaginal fistula.
Rectal stricture.
Vagina stenosis.
Surgery – Relatively few reports of use
In general, primary treatment with surgery is limited to early and small lesions confined to the vaginal mucosa (less than 2 cm).
Situations where surgery might be considered as first-line management are,
Patients presenting with stage I tumour in the upper third of the vagina; particularly posterior wall where
resection may be technically straightforward.
▪ Radical hysterectomy (1 cm disease-free margins)
▪ Pelvic lymphadenectomy
▪ Vaginectomy/ Colpectomy (1 cm disease-free margins)
1. Stage I upper third of vagina – Uterus has been removed
▪ Radical vaginectomy / Colpectomy as above with pelvic lymphadenopathy can be performed
2. Patients with small mobile stage I tumours low down in the vagina which is amenable to excision
▪ Vulvectomy with inguinal lymphadenectomy (1 cm disease-free margins)
3.
Central recurrence after radiation treatment
▪ Exenteration in a few careful selected cases.
4. Ovarian transposition/surgery pre-radiation
In young women with vaginal cancer requiring radiation as primary treatment, ovarian transposition can
be offered prior to definitive radiation treatment in an effort to prevent the adverse effects of
radiation-induced menopause.
▪
5. Laparoscopic or extraperitoneal removal of bulky lymph nodes can be offered as part of staging and treatment
planning.
6. Palliative management of recurrent or advanced disease
Advanced (Stage IV disease) or recurrent disease who present with vesicovaginal or rectovaginal
fistulae, a palliative urinary diversion or colostomy can be offered to improve quality of life
▪
7. Complications of surgery
May be frequent and serious
Urinary problems – Stress incontinence and/or urge incontinence.
Fistulae
Becoming apareunic
Chemotherapy
- There is very little published work.
Reports :
▪ Combined chemoradiation as first-line treatment of advanced disease
▪ Palliative use of chemotherapy for recurrent disease.
•
Survival - Overall 5-year survival rates are now in the region of 50% (39-66%)
- Survival rates for stage I disease consistently reported at between 70-80%
Prognostic factors
i. Stage
ii. Size
iii. Site
iv. Histological grade
v. Histological type
• - Vaginal melanoma has a very poor prognosis, with a five-year overall survival of 15%.
Vaginal Cancer Page 8
iii. Site
iv. Histological grade
v. Histological type - Vaginal melanoma has a very poor prognosis, with a five-year overall survival of 15%.
Recurrence - Occurs locally or within the pelvis in most instances.
- 20% relapsing with distant metastasis.
- The majority occurs soon after primary therapy.
Outcome:
▪ Is poor
▪ Further treatment is unlikely to be successful.
Vaginal melanoma
Vaginal melanoma is exceptionally rare and is typically diagnosed in elderly women.
The incidence is approximately three women per 10 million women per year
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