Vaginal Cancer

What is Vaginal Cancer?

Vaginal cancer is quite rare, making up only 1-2% of all cancers related to female reproductive organs. The vagina is a stretchy tube, about 7 to 10 centimeters long, that extends from the cervix (the lower part of the uterus) to the vulva (the outer part of the female genitalia). It’s nestled between the urethra, bladder, and the rectum. It’s divided into three parts and the location of these parts is important for identifying where a tumor is and where it might spread through the lymphatic system, which is a part of the immune system.

Vaginal cancer is specifically defined as a disease that shows no signs of cervical or vulvar cancer and the patient has had no history of either in the last five years. Most of the time, vaginal lesions or sores (80-90%) come from sores on the cervix or vulva, or from other nearby areas like the endometrium (the lining of the uterus), bladder, rectosigmoid (the lower part of the large intestine), or ovary. Occasionally, this cancer can spread to distant parts of the body, like the colon, breast, or pancreas. If vaginal cancer is suspected, a biopsy, which is a medical test involving taking a small sample of body tissue for examination, is done to confirm the diagnosis.

Vaginal cancer is estimated to be slightly more common than vulvar cancer, but these estimates include all genital sores, not just cancer. Because this type of cancer is so rare, there aren’t any dedicated studies devoted to finding proper treatment, so doctors usually follow guidelines based on a limited amount of research.

What Causes Vaginal Cancer?

Vaginal cancer can develop in two different ways. One way is induced by human papillomavirus (often abbreviated as HPV) and the other isn’t related to HPV. HPV, especially the strain called HPV 16, is often to blame for this type of cancer. The HPV induces the cancer by producing certain proteins which mess up normal cell functions. Specific proteins like E6 and E7 interfere with important cell proteins known as p53 and retinoblastoma protein (pRB), which usually help to control the growth and division of cells. These disturbances can contribute to the formation of a tumor.

In the past, a synthetic form of estrogen, named diethylstilbestrol (or DES for short), used to prevent complications during pregnancy, has been linked to a specific type of vaginal cancer (clear cell adenocarcinoma) in the daughters of women who used the drug. However, since DES stopped being regularly used in the 1970s, this type of cancer has become less common.

Risk Factors and Frequency for Vaginal Cancer

Squamous cell carcinoma (SCC) is the most common type of vaginal cancer, making up 90% of all cases. Other types of vaginal cancer include adenocarcinoma, clear cell adenocarcinoma, melanoma, sarcoma, and lymphoma.

Just like cervical cancer, vaginal cancer risk factors include smoking, starting sexual activity at a young age, HPV infection, and having multiple sexual partners.

The number of vaginal cancer cases that originate in the vagina tends to increase with age, with half of the patients being over 70 years old and 20% being over 80 years old.
SCC is the most common type of these cancers.
The average age of diagnosis is 67 years.
About 15% of women diagnosed with this cancer are under 50, and these cases are usually linked to cervical cancer.

Vaginal melanomas typically start from the mucosal melanocytes and are usually diagnosed due to vaginal bleeding. They are most commonly seen in white women around 60 years of age.

Verrucous carcinoma, a less dangerous variation of SCC, appears as a large, wart-like growth. Despite its aggressive growth in its originating area, it infrequently spreads to other parts of the body.

The most common type of sarcoma found in the vagina is embryonal rhabdomyosarcoma (ERMS) or sarcoma botryoides, a rare subtype of ERMS. These typically appear as soft, bumpy lesions and occur in infants and young children.

Signs and Symptoms of Vaginal Cancer

Vaginal cancer is a serious condition that requires careful consideration of medical and surgical history in addition to other potential signs. If a patient had other types of cancer before, especially cervical cancer, chances of getting vaginal cancer are higher. Often, the first sign of vaginal cancer is unusual bleeding from the vagina. Still, it’s important to note that 20% of women might not show any symptoms and can be unknowingly suffering from the disease. Other symptoms can include foul-smelling, bloody, or watery vaginal discharge or a lump in the vagina. When vaginal cancer starts to spread, it may cause urinary problems or gastrointestinal symptoms. Pelvic pain is usually a sign of advanced vaginal cancer.

Bleeding from the vagina which is typically irregular
No symptoms in certain women
Unusual vaginal discharge (foul-smelling, bloody, or watery)
A lump in the vagina
Urinary or gastrointestinal symptoms when the cancer spread
Pelvic pain, usually indicating advanced stage of the cancer

Evaluating potential cases of vaginal cancer involves several steps. There needs to be a complete physical exam, including tests that involve checking the rectum, vagina, and groin area for lumps. During a speculum exam, it’s important for the entire vagina to be viewed closely. Applying a solution containing iodine can assist in identifying potential cancerous cells. It’s also essential to verify if the patient has received the HPV vaccine, which can reduce the risk of developing vaginal cancer.

Testing for Vaginal Cancer

There aren’t any standard screenings for vaginal cancer. However, if a cervical test comes back abnormal and no issues are found in the cervix, it might be necessary to check for problems in the vagina. While regular testing for vaginal disease isn’t usually recommended after undergoing a hysterectomy (surgery to remove the uterus) for benign (non-cancerous) conditions, due to the risk of false positives, doctors do recommend long-term monitoring if you’ve had the procedure as a result of high-grade cervical intraepithelial neoplasia (an increase in abnormal cells on the surface of the cervix), particularly if you have a weakened immune system.

The treatment isn’t usually needed for low-grade vaginal intraepithelial neoplasia (VAIN), which is a precancerous condition in the vagina. It can be monitored using cytology (analysis of cells) and a colposcopy (a procedure to closely examine the cervix, vagina, and vulva).

The way to confirm the presence of vaginal cancer is through a biopsy (a procedure where a small sample of tissue is removed for examination). The best way to manage this is by carrying out the examination under anesthesia (when you’re sedated), including a thorough check of the vaginal fornices (the areas of the vagina extending up beside the cervix), and samples taken from the cervix. A clinical examination can also be conducted without sedation if this is more comfortable for you. If a primary invasive lesion is found (a significant growth that has invaded surrounding tissues), it becomes crucial to determine the extent or stage of the cancer to plan the treatment.

The current methods for staging include systems called the International Federation of Gynecology and Obstetrics (FIGO) staging, Tumor, Node, Metastasis (TNM) staging, and the American Joint Commission on Cancer (AJCC) staging. Plans for treatment may be modified based on results from imaging studies, such as MRI or CT scans.

In addition to physical examination, staging includes cystoscopy (a procedure to see inside your bladder and urethra), proctoscopy (a procedure to view the rectum and the end of the colon), chest X-rays, and bone scans. Also, testing of lymph nodes may be done, as well as examination of tissue removed during surgery. While there are no specific lab tests that can diagnose vaginal cancer, any significant rise in liver function can suggest the disease has spread but this isn’t definitive.

An MRI scan of the pelvis can be useful in assessing the stage of cancer in the vulva and vagina. It can give details about the size of the tumor, how far it has spread, and if it has spread to lymph nodes. An MRI scan can also help in diagnosing any changes after treatment and if the cancer has come back.

While imaging techniques such as CT, MRI, and positron emission tomography (PET) scans are helpful, they are not typically used to diagnose vaginal cancer. However, the International Federation of Gynecology and Obstetrics (FIGO) advises using these to guide treatment. According to one study, FIGO used PET/CT scans to check for suspected or known disease and found that in over half the cases, it changed both the expected outcome and the planned treatment.

It’s important to note that the vaginal lymphatic system, which helps the body fight infections, is very complex. The middle and upper vagina usually drain into the pelvic obturator node, internal and external iliac chains, and the para-aortic nodes. The lymph from the lower third of the vagina first drains into the groin lymph nodes and then into the pelvic nodes. The lymph from the back of the vagina drains with the anal lymphatics.

Treatment Options for Vaginal Cancer

Early-stage vaginal cancers are usually treated with surgery or radiation therapy. However, for advanced cancers, radiation therapy and chemotherapy are used together as surgery can be limited because the bladder, urethra, and rectum are close by. Occassionally, surgery may be useful in removing small tumors in their early stages or complex surgery can be considered if a fistula (an abnormal connection between two body parts) develops as a result of advanced or recurring disease post-radiation therapy.

For small lesions confined to the mucosa (the innermost lining of the organ), local excision may be possible. If the disease is higher up in the vagina, a radical hysterectomy (removal of the uterus), radical vaginectomy (removal of the vagina), and pelvic lymphadenectomy (removal of lymph nodes in the pelvic region) may be an option. For disease lower in the vagina, radical excision (removal of the affected area) with groin node dissection (removal of lymph nodes in the groin) is considered. The aim is to remove all detectable disease, aiming for disease-free margins (no remaining cancer cells) of 1 cm. While radical surgery for early-stage (stage I and II) cancers has shown promising results, these conclusions come from small, retrospective studies. Reports suggest that early-stage disease has an average five-year survival rate of 77%. In recurrent cases after radiation, pelvic exenteration (removal of all the organs of the pelvis) may be an option, but patients should be made aware of the significant risks and potential outcomes associated with this major surgery.

A study from the National Cancer Database points to the increased usage and effectiveness of combined chemoradiation therapy (CCRT) – a treatment plan combining chemotherapy and radiation therapy – for patients with vaginal cancer. Although the data is limited because vaginal cancer is not common, certain findings indicate that the use of combined therapy is beneficial for the patient’s survival. A standard approach for locally advanced vaginal cancers generally includes external beam radiation therapy (EBRT) which uses high-energy rays from outside the body, brachytherapy (internal radiation therapy), and weekly chemotherapy.

Vaginal melanoma, a rare type of vaginal cancer, does not have a standard treatment. However, surgery, and to an extent adjuvant therapy (additional cancer treatment given after the primary treatment to reduce the chance of cancer coming back) have been reported. Studies are being conducted with targeted therapies in vaginal melanoma; however, its genetic profile differs from other melanomas making treatment challenging.

The treatment of high-grade VAIN (vaginal intraepithelial neoplasia – a condition where abnormal cells are found on the surface of the vaginal lining that could potentially turn into cancer) can prevent it from turning into cancer. Various treatment modalities can be used, including laser ablation (using laser to remove tissue), surgical excision, and topical treatments such as imiquimod and topical chemotherapy with 5-fluorouracil. The choice of treatment is usually personalized to the patient’s specific condition.

When it comes to diagnosing vaginal cancer, doctors need to rule out many other conditions. These conditions might appear similar, but they are quite different. The following health issues may have similar symptoms or lead to complications that resemble vaginal cancer:

Sexually transmitted infections like herpes simplex and syphilis
Vaginal trauma and shrinkage
Non-cancerous growths in the vagina, such as polyps, Gartner duct cysts, Bartholin gland cysts, and vaginal adenosis
Other cancers in the reproductive system, including cervical and vulvar cancer
Bladder and colorectal cancer that has spread to the vagina

It’s vital for doctors to consider all these possibilities to ensure an accurate diagnosis.

Surgical Treatment of Vaginal Cancer

In the early stages of vaginal cancer (stage I), surgical treatment options can be considered. This could involve removal of the uterus (hysterectomy), upper part of the vagina (vaginectomy), and nearby lymph nodes. Ideally, the cancer should be located at the top of the back wall of the vagina. For cancer lower down, a more complex surgery involving the removal of the vagina and outer parts of the female genitals (vulva-vaginectomy) may be considered. However, this surgery is rare due to its complexity and potential complications.

For younger women whose vaginal cancer treatment includes radiation, moving the ovaries to a different location in the body (ovarian transposition) can be done before radiation treatment. This procedure can help prevent the onset of symptoms typically associated with menopause that can be triggered by radiation exposure.

In some patients, large lymph nodes can be removed through less invasive methods such as laparoscopic (small incision) or extraperitoneal (outside the lining of the abdominal cavity) surgery. This procedure can be performed for both staging (determining how far the cancer has spread) and creating a personalized treatment plan.

What to expect with Vaginal Cancer

Various factors can influence a patient’s treatment plan. An important factor is lymph node metastasis, which essentially means cancer has spread to the lymph nodes. Other factors that influence treatment include the type of cells involved in the cancer (histology), the size of the cancer, and the patient’s age. There was a study done by Surveillance, Epidemiology, and End Results (SEER), where they analyzed information of over 2000 patients.

According to their analysis, patients with stage I cancer had a five-year survival rate of 84%, while those with stage II had a 75% survival. However, survival rates decrease with advanced tumors, with a five-year survival rate of 57%. However, these are mere statistics and individual prognosis can greatly vary based on the specific circumstances of the patient.

Possible Complications When Diagnosed with Vaginal Cancer

The complications from treating vaginal cancer can come from a variety of sources and can be split into two categories: those that come from the treatment itself and those that depend on the patient’s own circumstances. Things like the intensity of radiation used, the kind of surgery performed, and the type of chemotherapy given are all factors connected to the treatment that could lead to complications. There are also factors tied to the patient like their age, hormone levels, and personal cleanliness habits that can influence the occurrence of complications.

Possible complications from radiation can include swelling (edema), skin redness (erythema) and inflammation of the mucous membranes (mucositis), which can potentially lead to ulcers. Generally, these effects clear up within a few months after treatment ends. For more information on managing side effects, see the section on Toxicity and Side Effect Management.

Complications List:

Swelling (Edema) from radiation
Skin redness (Erythema) from radiation
Inflammation of the mucous membranes (Mucositis) from radiation
Potential ulceration from radiation
Complications due to age, hormonal status, and personal hygiene
Complications due to intensity of radiation, type of surgery, and type of chemotherapy

Preventing Vaginal Cancer

A study looking at clinic visits found that female patients diagnosed with cancer may experience sexual and vaginal health issues that aren’t being fully addressed. The types of sexual health information these women prefer may change based on how old they are. Enhancing the conversation between doctors and patients about these issues, raising awareness, and providing “how-to” resources that use tested sexual health strategies can help women manage the side effects from their cancer treatment.

Frequently asked questions

Vaginal cancer is a rare disease that specifically refers to cancer in the vagina, which is the stretchy tube that extends from the cervix to the vulva. It is a type of cancer that shows no signs of cervical or vulvar cancer and has no history of either in the last five years.

Signs and symptoms of Vaginal Cancer include: - Irregular bleeding from the vagina - Some women may not show any symptoms - Unusual vaginal discharge, which can be foul-smelling, bloody, or watery - Presence of a lump in the vagina - Urinary or gastrointestinal symptoms when the cancer spreads - Pelvic pain, which usually indicates an advanced stage of the cancer It's important to note that 20% of women might not show any symptoms and can be unknowingly suffering from the disease. If any of these signs or symptoms are present, it is crucial to seek medical attention for further evaluation and diagnosis.

Vaginal cancer can be caused by human papillomavirus (HPV) or by factors unrelated to HPV, such as the use of diethylstilbestrol (DES) during pregnancy.

The doctor needs to rule out the following conditions when diagnosing Vaginal Cancer: - Sexually transmitted infections like herpes simplex and syphilis - Vaginal trauma and shrinkage - Non-cancerous growths in the vagina, such as polyps, Gartner duct cysts, Bartholin gland cysts, and vaginal adenosis - Other cancers in the reproductive system, including cervical and vulvar cancer - Bladder and colorectal cancer that has spread to the vagina

The types of tests that may be needed for diagnosing vaginal cancer include: 1. Cervical test: If an abnormality is found in the cervix, further testing may be necessary to check for problems in the vagina. 2. Cytology and colposcopy: These tests can be used to monitor low-grade vaginal intraepithelial neoplasia (VAIN), a precancerous condition in the vagina. 3. Biopsy: A biopsy is the most effective way to confirm the presence of vaginal cancer. It involves removing a small sample of tissue for examination. 4. Imaging studies: MRI or CT scans can be used to determine the extent or stage of the cancer and guide treatment. 5. Cystoscopy and proctoscopy: These procedures are used to examine the bladder, urethra, rectum, and colon for signs of cancer. 6. Chest X-rays and bone scans: These tests can help determine if the cancer has spread to other parts of the body. 7. Lymph node testing: Lymph nodes may be examined to check for the spread of cancer. 8. Liver function tests: A significant rise in liver function can suggest that the cancer has spread, although this is not definitive. 9. MRI scan of the pelvis: This scan can provide details about the size of the tumor, its spread, and whether it has spread to lymph nodes. 10. PET/CT scans: While not typically used for diagnosis, these scans can be used to guide treatment and may change the expected outcome and planned treatment. It's important to note that the specific tests ordered will depend on the individual patient and their specific condition.

Vaginal cancer can be treated with surgery, radiation therapy, chemotherapy, or a combination of these treatments. The specific treatment approach depends on the stage and location of the cancer. Early-stage vaginal cancers are usually treated with surgery or radiation therapy. For advanced cancers, radiation therapy and chemotherapy are used together, as surgery may be limited due to the proximity of nearby organs. In some cases, surgery may be used to remove small tumors or to address complications such as fistulas. The goal of treatment is to remove all detectable disease and achieve disease-free margins. Combined chemoradiation therapy (CCRT) has shown increased usage and effectiveness for vaginal cancer patients. Vaginal melanoma, a rare type of vaginal cancer, does not have a standard treatment, but surgery and adjuvant therapy have been reported. High-grade vaginal intraepithelial neoplasia (VAIN) can be treated with various modalities, including laser ablation, surgical excision, and topical treatments. Treatment options for vaginal cancer are personalized based on the individual patient's condition.

The side effects when treating Vaginal Cancer can include: - Swelling (Edema) from radiation - Skin redness (Erythema) from radiation - Inflammation of the mucous membranes (Mucositis) from radiation - Potential ulceration from radiation - Complications due to age, hormonal status, and personal hygiene - Complications due to intensity of radiation, type of surgery, and type of chemotherapy

The prognosis for vaginal cancer varies depending on the stage of the cancer. According to a study by Surveillance, Epidemiology, and End Results (SEER), patients with stage I cancer have a five-year survival rate of 84%, while those with stage II have a 75% survival rate. However, survival rates decrease with advanced tumors, with a five-year survival rate of 57%. It's important to note that individual prognosis can greatly vary based on the specific circumstances of the patient.

Gynecologic oncologist.

Cancer Diethylstilbestrol (DES) Human Papilloma Virus Human Papilloma Virus Vaccine