Table of Contents Purpose of This PDQ Summary General Information Stage Information
Treatment Option Overview Stage 0 Vaginal Cancer Stage I Vaginal Cancer Stage II Vaginal Cancer Stage III Vaginal Cancer Stage IVA Vaginal Cancer Stage IVB Vaginal Cancer Recurrent Vaginal Cancer Get More Information From NCI Changes to This Summary (05/22/2008) More Information
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of vaginal cancer. This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board 1.
Information about the following is included in this summary:
- Epidemiology.
- Prognosis.
- Cellular classification.
- Staging.
- Treatment options by cancer stage.
This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Some of the reference citations in the summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system 2 in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.
This summary is available in a patient version 3, written in less technical language, and in Spanish 4. General Information
Note: Estimated new cases and deaths from vaginal (and other female genital) cancer in the United States in 2008:[1]
- New cases: 2,210.
- Deaths: 760.
Carcinomas of the vagina are uncommon tumors comprising 1% to 2% of gynecologic
malignancies. They can be effectively treated, and when found in early stages,
are often curable. The histologic distinction between squamous cell carcinoma
and adenocarcinoma is important because the two types represent distinct
diseases, each with a different pathogenesis and natural history. Squamous
cell vaginal cancer (approximately 85% of cases) 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.[2] Adenocarcinoma (approximately 15% of cases) 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.[3] Rarely, melanoma and sarcoma are described as primary vaginal
cancers. Adenosquamous carcinoma is a rare and aggressive mixed epithelial
tumor comprising approximately 1% to 2% of cases.
Prognosis depends primarily on the stage of disease, but survival is reduced in
patients who are greater than 60 years of age, are symptomatic at the time of
diagnosis, have lesions of the middle and lower third of the vagina, or have
poorly differentiated tumors.[4,5] In addition, the length of vaginal wall
involvement has been found to be significantly correlated to survival and stage
of disease in squamous cell carcinoma patients.[6]
Therapeutic alternatives depend on stage; surgery or radiation therapy is
highly effective in early stages, while radiation therapy is the primary
treatment of more advanced stages.[7,8] Chemotherapy has not been shown to be
curative for advanced vaginal cancer, and there are no standard drug regimens.
Clear cell adenocarcinomas are rare and occur most often in patients less than
30 years of age who have a history of in utero exposure to diethylstilbestrol
(DES). The incidence of this disease, which is highest for those exposed
during the first trimester, peaked in the mid-1970s, reflecting the use of DES
in the 1950s.[3] Young women with a history of in utero DES exposure should
prospectively be followed carefully to diagnose this disease at an
early stage. In women who have been carefully followed and well-managed, the
disease is highly curable.
Vaginal adenosis is most commonly found in young women who had in utero
exposure to DES and may coexist with a clear cell adenocarcinoma, though it
rarely progresses to adenocarcinoma. Adenosis is replaced by squamous
metaplasia, which occurs naturally, and requires follow-up but not removal.
The natural history, prognosis, and treatment of other primary vaginal cancers
(sarcoma, melanoma, lymphoma, and carcinoid tumors) may be different, and
specific references should be sought.[9]
References
-
American Cancer Society.: Cancer Facts and Figures 2008. Atlanta, Ga: American Cancer Society, 2008. Also available online. 5 Last accessed October 1, 2008.
-
Gallup DG, Talledo OE, Shah KJ, et al.: Invasive squamous cell carcinoma of the vagina: a 14-year study. Obstet Gynecol 69 (5): 782-5, 1987.
[PUBMED Abstract]
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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 119 (5): 713-24, 1974.
[PUBMED Abstract]
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Kucera H, Vavra N: Radiation management of primary carcinoma of the vagina: clinical and histopathological variables associated with survival. Gynecol Oncol 40 (1): 12-6, 1991.
[PUBMED Abstract]
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Eddy GL, Marks RD Jr, Miller MC 3rd, et al.: Primary invasive vaginal carcinoma. Am J Obstet Gynecol 165 (2): 292-6; discussion 296-8, 1991.
[PUBMED Abstract]
-
Dixit S, Singhal S, Baboo HA: Squamous cell carcinoma of the vagina: a review of 70 cases. Gynecol Oncol 48 (1): 80-7, 1993.
[PUBMED Abstract]
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Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
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Pride GL, Schultz AE, Chuprevich TW, et al.: Primary invasive squamous carcinoma of the vagina. Obstet Gynecol 53 (2): 218-25, 1979.
[PUBMED Abstract]
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Sulak P, Barnhill D, Heller P, et al.: Nonsquamous cancer of the vagina. Gynecol Oncol 29 (3): 309-20, 1988.
[PUBMED Abstract]
Stage Information
Cervical biopsies are mandatory to rule out carcinoma of the cervix. Carcinoma
of the vulva should also be ruled out.
Stages are defined by the Federation Internationale de Gynecologie et
d’Obstetrique (FIGO) and the American Joint Committee on Cancer’s (AJCC) TNM
classification.[1] The definitions of the T categories correspond to the stages accepted by the FIGO and both systems are included for comparison.
TNM Definitions
TNM Categories/FIGO Stages
Primary tumor (T)
- TX: Primary tumor cannot be assessed
- T0: No evidence of primary tumor
- Tis/ 0: Carcinoma in situ
- T1/I: Tumor confined to vagina
- T2/II: Tumor invades paravaginal tissues but not to pelvic wall*
- T3/III: Tumor extends to pelvic wall*
- T4/IVA: Tumor invades mucosa of the bladder or rectum and/or extends beyond
the true pelvis (Bullous edema is not sufficient evidence to
classify a tumor as T4.)
* [Note: Pelvic wall is defined as muscle, facia, neurovascular structures, or skeletal portions of the bony pelvis.]
Regional lymph nodes (N)
- NX: Regional nodes cannot be assessed
- N0: No regional lymph node metastasis
- N1/IVB: Pelvic or inguinal lymph node metastasis
Distant metastasis (M)
- MX: Distant metastasis cannot be assessed
- M0: No distant metastasis
- M1/IVB: Distant metastasis
AJCC Stage Groupings
Stage 0
Stage I
Stage II
Stage III
- T1, N1, M0
- T2, N1, M0
- T3, N0, M0
- T3, N1, M0
Stage IVA
Stage IVB
References
-
Vagina. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 251-257.
Treatment Option Overview
Factors to be considered in planning therapy for vaginal cancer are:
- Stage, size, and location of the lesion.
- Presence or absence of the uterus.
- Whether there has been prior pelvic radiation therapy.
In a large series of women
studied retrospectively for 30 years, 50% had undergone hysterectomy prior to
the diagnosis of vaginal cancer.[1] In this posthysterectomy group, 31 of 50
(62%) women developed cancers limited to the upper third of the vagina. In women
who had not previously undergone hysterectomy, upper vaginal lesions were found
in only 17 of 50 (34%) women. The lymphatics may drain to pelvic or inguinal nodes
or both, depending on tumor location, and consideration should be given to
these areas in treatment planning. The proximity of the vagina to the bladder
or rectum limits treatment options and increases complications involving these
organs. For patients with carcinoma of the vagina in its early stages, standard treatment
applied by gynecologic oncologists or radiation oncologists 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
no established anticancer drugs can be considered standard treatment.
Considering the rarity of such patients, they should be considered candidates
for clinical trials using anticancer drugs and/or radiosensitizers to attempt
to improve survival or local control.
Information about ongoing clinical trials is available from the NCI Web site 6.
References
-
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 56 (1): 45-52, 1995.
[PUBMED Abstract]
Stage 0 Vaginal Cancer
Squamous Cell Carcinoma In Situ
This disease is usually multifocal and commonly occurs at the vaginal vault.
Because vaginal intraepithelial neoplasia (VAIN) is associated with other
genital neoplasias, the cervix (when present) and vulva should be carefully
examined. The treatments listed below produce equivalent cure rates. The
selection of treatment depends on patient factors and local expertise (e.g.,
anatomical distortion of the vaginal vault [related to wall closure at the time
of hysterectomy] requires excision for technical reasons to exclude the
possibility of invasion by buried disease). Lesions with hyperkeratosis respond
better to excision or laser vaporization than to fluorouracil.[1]
Standard treatment options:
- Wide local excision with or without skin grafting.
- Partial or total vaginectomy with skin grafting for multifocal or extensive
disease.
- Intravaginal chemotherapy with 5% fluorouracil cream. Instillation of 1.5
g weekly for 10 weeks has been found to be as effective as more frequent
use.[2]
- Laser therapy.[2]
- Intracavitary radiation therapy delivering 60 Gy to 70 Gy to the
mucosa.[3,4] The entire vaginal mucosa should be treated.[5]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage 0 vaginal cancer 7. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
Wright VC, Chapman W: Intraepithelial neoplasia of the lower female genital tract: etiology, investigation, and management. Semin Surg Oncol 8 (4): 180-90, 1992 Jul-Aug.
[PUBMED Abstract]
-
Krebs HB: Treatment of vaginal intraepithelial neoplasia with laser and topical 5-fluorouracil. Obstet Gynecol 73 (4): 657-60, 1989.
[PUBMED Abstract]
-
Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
-
Woodman CB, Mould JJ, Jordan JA: Radiotherapy in the management of vaginal intraepithelial neoplasia after hysterectomy. Br J Obstet Gynaecol 95 (10): 976-9, 1988.
[PUBMED Abstract]
-
Perez CA, Garipagaoglu M: Vagina. In: Perez CA, Brady LW, eds.: Principles and Practice of Radiation Oncology. 3rd ed. Philadelphia, Pa: Lippincott-Raven Publishers, 1998, pp 1891-1914.
Stage I Vaginal Cancer
Squamous Cell Carcinoma
The treatments listed below produce equivalent cure rates. The
selection of treatment depends on patient factors and local expertise.
Standard treatment options for superficial lesions less than 0.5 cm thick:
- Intracavitary radiation therapy. In most instances, 60 Gy to 70 Gy
prescribed to 0.5 cm is delivered to the tumor for 5 to 7 days
(external-beam radiation therapy [EBRT] is required for bulky lesions).[1] For lesions of
the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy
is given to pelvic and/or inguinal lymph nodes.[1]
- Surgery. Wide local excision or total vaginectomy with vaginal
reconstruction, especially in lesions of the upper vagina. In cases with
close or positive surgical margins, adjuvant radiation therapy should be
considered.[2]
Standard treatment options for lesions greater than 0.5 cm thick:
- Surgery. In lesions of the upper third of the vagina, radical
vaginectomy and pelvic lymphadenectomy should be performed. Construction of a
neovagina may be performed if feasible and if desired by the patient.[2,3]
In lesions of the lower third, inguinal lymphadenectomy should be
performed. In cases with close or positive surgical margins, adjuvant
radiation therapy should be considered.[2]
- Radiation therapy. Combination of interstitial (single-plane implant) and
intracavitary therapy to a dose of at least 75 Gy to the primary tumor.
In addition to brachytherapy, EBRT is advocated for
poorly differentiated or infiltrating tumors that may have a higher
probability of lymph node metastasis.[1,4] For lesions of the lower third of
the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to the
pelvic and/or inguinal lymph nodes.[1]
Adenocarcinoma
Standard treatment options:
- Surgery. Because the tumor spreads subepithelially, total radical
vaginectomy and hysterectomy with lymph node dissection are indicated. The
deep pelvic nodes are dissected if the lesion invades the upper vagina, and the
inguinal nodes are removed if the lesion originates in the lower vagina.
Construction of a neovagina may be performed if feasible and if desired by the
patient.[2] In cases with close or positive surgical margins, adjuvant
radiation therapy should be considered.[2,3]
- Intracavitary and interstitial radiation as previously described for
squamous cell cancer.[1] For lesions of the lower third of the vagina,
elective radiation therapy of 45 Gy to 50 Gy is given to the pelvic and/or inguinal
lymph nodes.[1]
- Combined local therapy in selected cases, which may include wide local
excision, lymph node sampling, and interstitial therapy.[5]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I vaginal cancer 8. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
-
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 56 (1): 45-52, 1995.
[PUBMED Abstract]
-
Rubin SC, Young J, Mikuta JJ: Squamous carcinoma of the vagina: treatment, complications, and long-term follow-up. Gynecol Oncol 20 (3): 346-53, 1985.
[PUBMED Abstract]
-
Andersen ES: Primary carcinoma of the vagina: a study of 29 cases. Gynecol Oncol 33 (3): 317-20, 1989.
[PUBMED Abstract]
-
Senekjian EK, Frey KW, Anderson D, et al.: Local therapy in stage I clear cell adenocarcinoma of the vagina. Cancer 60 (6): 1319-24, 1987.
[PUBMED Abstract]
Stage II Vaginal Cancer
Squamous Cell Carcinoma
Radiation therapy is the standard treatment for patients with stage II vaginal carcinoma.
Standard treatment options:
- Combination of brachytherapy and external-beam radiation therapy (EBRT) to deliver
a combined dose of 70 Gy to 80 Gy to the primary tumor volume.[1] For
lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50
Gy is given to the pelvic and/or inguinal lymph nodes.[1,2]
- Radical surgery (radical vaginectomy or pelvic exenteration) with or
without radiation therapy.[3,4]
Adenocarcinoma
Standard treatment options:
- Combination of brachytherapy and EBRT to deliver
a combined dose of 70 Gy to 80 Gy to the primary tumor.[1] For lesions of
the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is
given to the pelvic and/or inguinal lymph nodes.[1,2]
- Radical surgery (radical vaginectomy or pelvic exenteration) with or without
radiation therapy.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II vaginal cancer 9. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
-
Andersen ES: Primary carcinoma of the vagina: a study of 29 cases. Gynecol Oncol 33 (3): 317-20, 1989.
[PUBMED Abstract]
-
Rubin SC, Young J, Mikuta JJ: Squamous carcinoma of the vagina: treatment, complications, and long-term follow-up. Gynecol Oncol 20 (3): 346-53, 1985.
[PUBMED Abstract]
-
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 56 (1): 45-52, 1995.
[PUBMED Abstract]
Stage III Vaginal Cancer
Squamous Cell Carcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and external-beam radiation
therapy (EBRT). EBRT for a period of 5 to 6 weeks (including pelvic
nodes) followed by an interstitial and/or intracavitary implant for a total
tumor dose of 75 Gy to 80 Gy and a dose to the lateral pelvic wall of 55
Gy to 60 Gy.[1]
- Rarely, surgery may be combined with the above.[2]
Adenocarcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and EBRT as described for squamous cell cancer.[1]
- Rarely, surgery may be combined with the above.[2]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III vaginal cancer 10. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
-
Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987.
[PUBMED Abstract]
Stage IVA Vaginal Cancer
Squamous Cell Carcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and external-beam radiation
therapy (EBRT).[1]
- Rarely, surgery may be combined with the above.[2]
Adenocarcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and EBRT.[1]
- Rarely, surgery may be combined with the above.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IVA vaginal cancer 11. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.
[PUBMED Abstract]
-
Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987.
[PUBMED Abstract]
Stage IVB Vaginal Cancer
Squamous Cell Carcinoma
Patients should be considered candidates for one of the ongoing clinical trials
to improve therapeutic results. Standard treatment is inadequate.
Standard treatment options:
- Radiation (for palliation of symptoms) with or without chemotherapy.
Adenocarcinoma
Patients should be considered candidates for one of the ongoing clinical trials
to improve therapeutic results.
Standard treatment options:
- Radiation (for palliation of symptoms) with or without chemotherapy.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IVB vaginal cancer 12. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
Recurrent Vaginal Cancer
Recurrence carries a grave prognosis. In a large series only five of fifty
patients with recurrence were salvaged by surgery or radiation therapy. All
five of these salvaged patients originally presented with stage I or II disease
and failed in the central pelvis.[1] Most recurrences are in the first 2 years
after treatment. In centrally recurrent vaginal cancers, some patients may be
candidates for pelvic exenteration or radiation therapy. Neither cisplatin nor mitoxantrone has
significant activity in recurrent or advanced squamous cell cancer. There is
no standard chemotherapy.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent vaginal cancer 13. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site 6.
References
-
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 56 (1): 45-52, 1995.
[PUBMED Abstract]
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The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary. More Information
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