Vaginal cancer is an extraordinarily rare form of

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...

that develops in the tissue of the

vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hymen ...

. Primary vaginal cancer originates from the vaginal tissue – most frequently squamous cell carcinoma, but primary vaginal adenocarcinoma, sarcoma, and melanoma have also been reported – while secondary vaginal cancer involves the metastasis of a cancer that originated in a different part of the body. Secondary vaginal cancer is more common. Signs of vaginal cancer may include abnormal vaginal bleeding,

dysuria Dysuria refers to painful or uncomfortable urination. It is one of a constellation of ''irritative'' bladder symptoms (also sometimes referred to as lower urinary tract symptoms), which includes nocturia and urinary frequency. Diagnosis The c ...

, tenesmus, or

pelvic pain Pelvic pain is pain in the area of the pelvis. Acute pain is more common than chronic pain. If the pain lasts for more than six months, it is deemed to be chronic pelvic pain. It can affect both the male and female pelvis. Common causes in include ...

, though as many as 20% of women diagnosed with vaginal cancer are asymptomatic at the time of diagnosis. Vaginal cancer occurs more frequently in women over age 50, and the mean age of diagnosis of vaginal cancer is 60 years. It often can be cured if found and treated in early

stages Stage or stages may refer to: Acting * Stage (theatre), a space for the performance of theatrical productions * Theatre, a branch of the performing arts, often referred to as "the stage" * ''The Stage'', a weekly British theatre newspaper * S ...

. Surgery alone or surgery combined with pelvic

radiation In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or through a material medium. This includes: * ''electromagnetic radiation'', such as radio waves, microwaves, infrared, visi ...

is typically used to treat vaginal cancer.

Description

Carcinoma of the vagina occurs in less than 2% of women with pelvic malignant tumors. Squamous carcinoma is the most common type of vaginal cancer. The human papilloma virus (HPV) is strongly associated with vaginal cancer. Vaginal cancer occurs most often in the upper third of the vagina (51%), 30% are found in the lower third, and 19% in the middle third. Vaginal cancer can present as an elevated lesion growing out from the epithelial surface or an ulcer-like, shallow depression. Definitive diagnosis is determined by biopsy.

Signs and symptoms

Most vaginal cancers do not cause signs or symptoms early on. When vaginal cancer does cause symptoms, they may include: * Vaginal discharge or abnormal bleeding * Unusually heavy flow of blood * Bleeding after menopause * Bleeding between periods; or any other * Bleeding that is longer than normal * Blood in the stool or urine * Frequent or urgent need to urinate * Feeling constipated * Pain during sexual intercourse * A lump or growth in the vagina that can be felt. Enlarged pelvic lymph nodes can sometimes be palpated.

Risk factors

* Prenatal exposure to

diethylstilbestrol Diethylstilbestrol (DES), also known as stilbestrol or stilboestrol, is a nonsteroidal estrogen medication, which is presently rarely used. In the past, it was widely used for a variety of indications, including pregnancy support for those with a ...

* Infection with human papillomavirus (HPV) type 16 * Infection with human immunodeficiency virus (HIV) type 1 * Previous history of cervical cancer * Smoking * Chronic vulvar itching or burning

Types

There are two primary types of vaginal cancer:

squamous-cell carcinoma Squamous-cell carcinomas (SCCs), also known as epidermoid carcinomas, comprise a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the ...

and adenocarcinoma. *

Squamous-cell carcinoma of the vagina Squamous-cell carcinoma of the vagina is a potentially invasive type of cancer that forms in the tissues of the vagina. Though uncommon, squamous-cell cancer of the vagina (SCCV) is the most common type of vaginal cancer. It is further subdivide ...

arises from the

squamous cell Epithelium or epithelial tissue is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. It is a thin, continuous, protective layer of compactly packed cells with a little intercellula ...

s (epithelium) that line the vagina. This is the most common type of vaginal cancer. It is found most often in women aged 60 or older. * Vaginal

adenocarcinoma Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) (AC) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or ...

arises from the glandular (secretory) cells in the lining of the vagina. Adenocarcinoma is more likely to spread to the

lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...

s and

lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inclu ...

s. * Clear cell adenocarcinoma occurs in a small percentage of women (termed "DES-Daughters") born between 1938 and 1973 (later outside the United States) that were exposed to the drug

(DES) in utero. DES was prescribed to 5 to 10 million mothers period to prevent possible miscarriages and premature births. Typically, women develop DES-related adenocarcinoma before age 30, but increasing evidence suggests possible effects or cancers (including other forms of vaginal glandular tumors) at a later age. DES-exposure in women is also linked to various infertility and pregnancy complications. Daughters exposed to DES in utero may also have an increased risk of moderate/severe cervical squamous cell dysplasia and an increased risk of breast cancer. Approximately one in 1,000 (0.1%) DES daughters will be diagnosed with clear cell adenocarcinoma. The risk is virtually non-existent among premenopausal women not exposed to DES. * Vaginal germ cell tumors (primarily

teratoma A teratoma is a tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the ovary, testicle, or coccyx. Symptoms Symptoms may be minimal if the tumor is small. A testicular ter ...

and

endodermal sinus tumor Endodermal sinus tumor (EST) is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under three, and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contr ...

) are rare. They are found most often in infants and children. * Sarcoma botryoides, a

rhabdomyosarcoma Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. There are four subt ...

also is found most often in infants and children. *

Vaginal melanoma Vaginal melanoma is a rare malignancy that originates from Melanocyte, melanocytes in the vaginal epithelium. It is also known as a melanocytic tumor or as a malignant melanoma. It is aggressive and infrequently cured. The median overall survival i ...

, a

melanoma Melanoma, also redundantly known as malignant melanoma, is a type of skin cancer that develops from the pigment-producing cells known as melanocytes. Melanomas typically occur in the skin, but may rarely occur in the mouth, intestines, or eye ( ...

that appears in the vagina. File:Diagram showing stage 1 vaginal cancer CRUK 194.svg, Stage 1 vaginal cancer File:Diagram showing stage 2 vaginal cancer CRUK 210.svg, Stage 2 vaginal cancer File:Diagram showing stage 3 vaginal cancer CRUK 223.svg, Stage 3 vaginal cancer File:Diagram showing stage 4A vaginal cancer CRUK 237.svg, Stage 4A vaginal cancer File:Diagram showing stage 4B vaginal cancer CRUK 240.svg, Stage 4B vaginal cancer

Diagnosis and Screening

Routine vaginal cancer screening, including routine surveillance imaging such as ultrasound or MRI, is not recommended for women who do not have symptoms. Imaging without indications is discouraged because it is unlikely to detect a recurrence or improve survival, and because it has its own costs and side effects. Several tests are used to diagnose vaginal cancer, including: * Physical exam and history *

Pelvic exam A pelvic examination is the physical examination of the external and internal female pelvic organs. It is frequently used in gynecology for the evaluation of symptoms affecting the female reproductive and urinary tract, such as pain, bleeding, d ...

* Biopsy *

Colposcopy Colposcopy ( grc, κόλπος, kolpos, hollow, womb, vagina + ''skopos'' "look at") is a medical diagnostic procedure to visually examine the cervix as well as the vagina and vulva using a colposcope. The main goal of colposcopy is to prevent c ...

MRI provides visualization of the extent of vaginal cancer. Other sources of cancer tissue such as the urethra or the cervix must be ruled out before a diagnosis of vaginal cancer is made. Vaginal cancer cannot be detected through cervical pap smears.

Classification

Staging: The

International Federation of Gynecology and Obstetrics The International Federation of Gynaecology and Obstetrics, usually just FIGO ("fee'go") as the acronym of its French name Fédération Internationale de Gynécologie et d'Obstétrique, is a worldwide non-governmental organisation representing ob ...

utilizes the Tumor, Node, Metastasis (TNM) method of staging vaginal cancer. The clinical staging of the most common form of primary vaginal cancer, squamous cell carcinoma, is outlined below. * Stage I - Tumor confined to the vagina * Stage II - Tumor invading tissue near the vagina, but has not spread to pelvic wall * Stage III- Tumor invading pelvic sidewall or interfering with renal function, obstructing ureter to cause hydronephrosis. * Stage IV - Tumor invading mucosa of nearby pelvic organs, including bladder or rectum, or has spread beyond pelvis.

Management

Historically, the combination of external-beam

radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

(EBRT) has been the most common treatment for vaginal cancer. In early stages of vaginal cancer, surgery also has some benefit. This management and treatment is less effective for those with advanced stages of cancer but works well in early stages with high rates of cure. Advanced vaginal cancer only has a 5-year survival rates of 52.2%, 42.5% and 20.5% for patients with stage II, III and IVa disease. Newer treatments for advanced stages of ovarian have been developed. These utilize concurrent carboplatin plus paclitaxel, EBRT and high-dose-rate interstitial brachytherapy (HDR-ISBT). When the chance of surgical removal of all cancerous tissue is very low or when the surgery has a chance of damaging the bladder, vagina or bowel, radiation therapy is used. When a tumor is less than 4 cm in diameter, radiation therapy provides excellent results. In these instances, the 5-year survival rate is greater than 80%. Treatments are individualized due to the rarity of vaginal cancer studies. File:Diagram showing a wide local excision for vaginal cancer CRUK 087.svg, A local surgery to remove vaginal cancer File:Diagram showing a radical hysterectomy for vaginal cancer CRUK 075.svg, A radical hysterectomy to treat vaginal cancer without reconstruction File:Diagram showing a radical hysterectomy with a reconstructed vagina CRUK 076.svg, A radical hysterectomy for vaginal cancer with reconstruction of the vagina using other tissues

Epidemiology

Cancer of the vagina is rare and is only 2% of all gynecological cancers and less than 0.5% of all cancers in women. Estimated new cases in the United States in 2017 are 4,810. Deaths from vaginal cancer during the same time were 1,240. It is more common in older women. In the UK, 254 cases of vaginal cancer were identified in 2014. Deaths from vaginal cancer in this period were 110. Out of those with vaginal cancer, 53% are related to HPV infection.

Research

Clinical trials

* Because vaginal cancer is so rare, there have been very few phase three clinical trials studying treatments specifically targeting vaginal cancer. There is an ongoing phase three trial studying the efficacy of the drug

triapine 3-Aminopyridine-2-carboxaldehyde thiosemicarbazone (3-AP, also called Triapine) is a substance that is being studied in the treatment of cancer. It belongs to the family of drugs called ribonucleotide reductase inhibitors. 3AP is a potent inhib ...

to treat vaginal cancer. There are currently several phase one and two clinical trials underway to study the efficacy of new gynecological cancer treatment regimens, which include treatment for vaginal cancer.

References

External links

Clearinghouse of Information for DES daughters and Sons

National Cancer Institute: Vaginal Cancer

Clinically reviewed vaginal cancer information for patients

UK vaginal cancer statistics

Cancer.Net: Vaginal Cancer''Vagina'', Anatomical Atlases, an Anatomical Digital Library (2018)
{{DEFAULTSORT:Vaginal Cancer Gynaecological cancer Papillomavirus-associated diseases Vagina Women's health Oncology Rare cancers