Vaginal Neoplasia

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Vaginal Neoplasia

Howard Kent, MD

Introduction

Vaginal neoplasia was first described in 1877 by Cruveilhier
Vaginal intraepithelial neoplasia (VaIN) was first described by Hummer in 1933
Vaginal carcinoma is rare (rate 0.5/100,000)
Intraepithelial carcinoma is even rarer than vaginal carcinoma
Neoplastic lesions of the vagina are far less frequent than corresponding lesions in either the vulva or cervix

Incidence

Squamous vaginal carcinoma is typically a disease of older women. Seventy percent of cases occur in women past the age of 50. The peak incidence is between age 60 & 70.
In women who have had a surgically created neovagina, the incidence peaks at a much younger age - between 25 and 35.
While little is known about the epidemiology of vaginal carcinoma, there is evidence that radiotherapy given earlier for other gyn malignancies may increase the risk. If the period between application of radiation to the development of vaginal cancer is greater than 10 years, the prognosis is much improved.
Factors that may increase the risk of vaginal carcinoma may include: human papillomavirus infection, immunosuppression, and chronic irritation from pessary use.
Primary carcinoma of the vagina must be differentiated from vaginal extension from either the vulva or the cervix, and from metastatic extension of other tumors.
A valid diagnosis of a primary vaginal cancer necessitates that there be no cancer of the vulva or the cervix for at least 10 years or more prior to that diagnosis

Staging System for Carcinoma of the Vagina - FIGO nomenclature

Stage	Clinical Status
0	Carcinoma in situ
I	The carcinoma is limited to the vaginal wall
II	The carcinoma has involved the subvaginal tissue but has not extended to the pelvic wall
III	The carcinoma has extended to the pelvic wall
IV	The carcinmoma has extended beyond the true pelvis or has involved the mucosa of the bladder or rectum; bullous edemas as such does not permit a case to be allotted to Stage IV
IVa	Tumor invades bladder and/or rectal mucosa and/or direct extension beyond the true pelvis
IVb	Spread to distant organs

Note:

There is current discussion on including urethral mucosal involvement
Melanoma is staged using Breslow depth system

Pathology

Only 10-20% of vaginal carcinomas are primary
Squamous cell carcinoma is the most common malignant tumor of the vagina
Clear cell adenocarcinoma is found in women who had exposure to diethylstilbesterol in utero and to a lesser degree in postmenopausal individuals
Classification of intraepithelial neoplasias of the vagina parallels that of the cervix (e.g., VaIN 1, VaIN2, and VaIN3
VaIN is usually asymptomatic and is diagnosed by abnormal cytologic testing (infrequently women complain of postcoital staining or unusual vaginal discharge)
VaIN usually occurs in the upper third of the vagina on the posterior wall
VaIN lesions may be either single, discrete, or multifocal

Colposcopic Evaluation

VaIN is most frequently diagnosed by colposcopy
Care must be exercised to make sure the entire surface area of the vagina is examined, including that behind the blades of the speculum
In post hysterectomy patients, the lateral invagination of the vaginal vault must be carefully evaluated. The rugate pattern must be flattened out so that complete inspection can be done.
3-5% acetic acid is applied to the vaginal walls for 3 minutes to detect acetowhitening
Lugol’s iodine solution is then applied. Normal tissue will stain mahogany while tumor cells will not retain the color.
Areas of VaIN are typically discrete with slightly elevated borders and are pinkish or white in color
VaIN has typically the same patterns as those of CIN, but after the application of acetic acid the appearance of the lesion is often more subtle and less easily detected
VaIN appears as an area of acetowhite epithelium
VaIN may have a well established vascular pattern characterized by punctation and mosaicism
The vascular pattern associated with high grade VaIN occurs late in the neoplastic process and is characterized by bizarre vascular forms
Careful biopsy of suspicious areas is necessary to finalize the diagnosis

Treatment of VaIN

VaIN tends to be multifocal and associated with HPV (by contrast, most invasive cancers are unifocal and are less often associated with HPV)
VaIN tends to occur in younger patients
Response of VaIN is unpredicatable - in some cases it regresses, in some it persists, while in some it progresses (probably less than 10%) to invasive cancer
Minor lesions probably can be carefully followed and are probably viral in origin
High grade lesions should be treated.

Treatment

Treatment options should take in consideration the following data:
- age of the patient
- sexual activity of the patient
- extent of the lesion
- previous history of radiotherapy
- presence or absence of the cervix
- grade of the lesion
- site of the lesion
If invasion is suspected, surgical excision, with or without skin grafting is the treatment of choice. Cure rates approach 90%.
Low-grade lesions generally do not require treatment
CO₂ laser can be used to vaporize discrete lesions that are high-grade, but care must be exercised
Vaginal invasive carcinoma is treated with vaginectomy or radiation therapy.

Other Vaginal Epithelial Tumors of Lesser Significance

Verrucous carcinoma - very rare - resembles condylomata. May recur after surgical excision but distant metastases are rare
Basal cell carcinoma - very rare - similar to vulvar basal cell carcinoma
Adenocarcinoma - extremely rare lesion prior to the DES-exposure era. Can occur in any area of the vagina, but is most common in the upper third on the anterior or posterior wall.

Sarcoma

Sarcomas comprise less than 2% of all malignant neoplasms. Of these, embryonal rhabdomyosarcoma (sarcoma botryoides) is the most common type in infants and young adolescent girls
Many occur prior to the age of 5 (2/3 within the first 2 years)
Infiltrates the vaginal wall and pelvis, and presents as a polypoid mass of tissue resembling grapes
The five year survival is between 10 and 35%
Pelvic extenteration is the treatment of choice

Melanoma

Is the second most common cancer of the vagina - 3% of vaginal malignancies (0.3% of all melanomas)
Usually occurs in the lower third of the vagina
Frequently black or blue pigmentation
Lesions are frequently ulcerated
Amelanotic lesions can occur. Immunoperoxidase staining for S-100 protein can facilitate the diagnosis

Metastatic Tumors

Endometrial and cervical are the most common metastases to the vagina
Tumors of the ovary, rectum, and kidney can also spread to the vagina
Choriocarcinoma can also metastasize to the vagina

References

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