All About Vulvar Cancer

Neha Vapiwala, MD, Eric T. Shinohara, MD, MSCI, and Carolyn Vachani, RN, MSN, AOCN
Updated by: Christina Bach, MBE, MSW, LCSW, OSW-C
The Abramson Cancer Center of the University of Pennsylvania
Last Modified: September 19, 2016

What is vulvar cancer?

Vulvar cancer is an abnormal growth of malignant (cancerous) cells in the vulva. The vulva is defined as the external female genitalia, and includes the labia majora (outer lips of labia), labia minora (inner lips), clitoris, mons pubis, vestibule, or entryway, of the vagina, and the perineum (area between vulva and anus).

About 80% of vulvar cancers involve the labia, mainly the labia majora (~50%). About ten percent involve the clitoris, and another 10% involve the perineum, which is the area of sensitive skin located between the vagina and the anus. In about 5% of cases, the cancer is present at more than one site.

What are the different types of vulvar cancer?

The vulva is essentially epithelial skin; therefore the main tumor types that affect the vulva are skin-related cancers.

About 90% of vulvar cancers are squamous cell carcinomas, which typically develop at the edges of the labia majora/ minora or in the vagina. As with vaginal squamous cell carcinomas, vulvar squamous cell cancers are slow growing and usually develop from "precancerous", pre-invasive areas called vulvar intraepithelial neoplasia (VIN). There are two subtypes of squamous cell vulvar cancer. One is more common in younger women and is associated with the human papillomavirus (HPV); the other occurs in older women and is not associated with HPV infection, but is associated with chronic vulvar skin changes called vulvar dystrophy, including lichen sclerosus.

Melanoma is the second most common type of cancer found in the vulvar area and represents less than 5% of vulvar cancer cases. The most common skin-cancer in sun-exposed areas is basal cell carcinoma, and as expected, this type rarely occurs on the vulva.

A sarcoma of the soft tissue can develop in the vulva and these account for 1-2% of vulvar cancers. Adenocarcinomas of the vulva are also rare, but can develop from glands, most commonly the Bartholin's glands located at the vaginal opening.

Am I at risk for vulvar cancer?

Vulvar cancer is a relatively rare cancer, representing about 4% of all gynecologic cancers, and only about .6% of all female cancers in general. The American Cancer Society estimates that in 2016 there will be 5,950 new cases of vulvar cancer reported in the US and approximately 1,100 deaths a year are attributed to this disease.

Vulvar cancer most commonly occurs in postmenopausal women. The peak age of diagnosis is between 70-79 years old. However, the rate of vulvar cancers diagnosed in younger women has been increasing in the last decade, as a result of vulvar cancers caused by the Human Papilloma Virus (HPV) infection. These HPV-associated vulvar cancers are often seen in women under 45 years of age. Experts agree that these two groups of women have different types of squamous cell vulvar cancer, which behave differently and respond differently to treatment.

In post-menopausal women, vulvar cancer is often associated with long-term changes in the vulvar skin (vulvar dystrophy, squamous cell hyperplasia or lichen sclerosus). This may be a thickening or thinning of the vulvar skin or a white area that may be itchy or painful. Older women are less likely to attend preventive gynecologic healthcare visits and these conditions may go undiagnosed.

HPV Associated Vulvar Cancers

More than half of vulvar cancers are caused by HPV infection. HPV is a sexually transmitted disease that is very common in the population. Most college-aged men and women have been exposed to HPV, though in most, the immune system inactivates or clears the virus from the body. There are over 100 different subtypes, or strains, of HPV and only certain subtypes are "oncogenic", or able to cause cancer (these include: HPV 16,18, 33, 39). Certain strains of HPV cause genital warts, though these strains are not oncogenic. Infection with HPV typically causes no symptoms, and may only be detected when a woman has an abnormal pap result or HPV testing that may be done along with the pap test. It is important to know that only a very small percentage of women who have a high-risk strain of HPV will develop a cancer caused by the virus; so simply having HPV does not mean that you will get cancer.

Women who have had multiple male sexual partners, began having sexual intercourse at an early age, or have had male sexual partners who are considered high risk (meaning that they have had many sexual partners and/or began having sexual intercourse at an early age) are at a higher risk for developing an HPV-related cancer.

HPV-associated vulvar cancers may appear in more than one location and may occur in conjunction with cervical, vaginal or perianal cancers, as these are also caused by HPV infection. 

Being a current smoker is also considered a risk factor. Smoking is linked to an inability for the body's immune system to clear an HPV infection; therefore, smokers are more likely to develop chronic HPV infections that may lead to a cancer.

HIV infection or a history of other gynecologic cancer or melanoma can also increase the risk of developing vulvar cancer.

How can I prevent vulvar cancer?

It may be possible to lower your risk of developing vulvar cancer by avoiding HPV exposure, and by stopping or never starting to smoke.  Pap smear/pelvic examination may pick up early signs of vulvar cancer, so having this exam performed regularly is suggested (every 3-5 years depending on age and HPV status). 

What are the symptoms of vulvar cancer?

The classic symptom is vulvar itching (pruritus), reported in almost 90% of the women with vulvar cancer. There can also be associated pain, pain with intercourse, bleeding, vaginal discharge, and/or painful urination (dysuria). Pre-cancerous lesions or early vulvar cancers may have mild or minimal symptoms. Preventive gynecologic exams can be helpful in detecting these early lesions.

Ultimately, many women will develop a visible vulvar mass: the squamous cell subtype can look like elevated white, pink or red bumps, while vulvar melanoma characteristically presents as a colored, ulcerated growth. There can be portions of the tumor that look sore and scaly, or cauliflower-like (similar to HPV-related warts).

How is vulvar cancer diagnosed?

First and foremost, a thorough gynecological examination should be performed using colposcopy and/or vulvoscopy, which uses a special magnifying instrument for better visualization. Any abnormal-appearing area(s) should be biopsied. Up to one-half of the time, the cancer may be "multi-focal"; meaning that the cancer is in two separate places. In addition, other HPV-related cancers can also be present (i.e. cervical, vaginal, perianal). Hence, a careful exam of all of the skin in the vaginal and groin area, as well as a gynecological exam should be performed. A Pap smear should be performed and additional smears taken from the vagina and vulva for testing.

CT scan or MRI of the abdomen/pelvis may be done to look for disease spread to lymph nodes and/or distant organs in advanced cases, but is not necessary in early stage disease. If spread to bladder or rectum is suspected, endoscopy (a scope to evaluate these areas; cystoscopy and proctoscopy, respectively) should be performed.

How is vulvar cancer staged?

Vulvar cancer can spread by direct extension, meaning that they can grow into adjacent areas such as the vagina and anus. Even in early disease, spread to lymph nodes can occur. However, spread to other organs is rare until late in the course of the disease.

Once a diagnosis is confirmed, vulvar cancer is staged. Staging helps the healthcare team better understand the prognosis and decide which treatment options are best for each individual. 

There are two staging systems for vulvar cancer. The first is the FIGO system (International Federation of Gynecologists and Obstetricians). The AJCC TNM system (also called the tumor-node-metastasis system) describes the size and local invasiveness of the tumor (T), which, if any lymph nodes are involved (N), and if it has spread to other more distant areas of the body (M). This is then interpreted as a stage between I (one) denoting limited disease to IV (four) denoting more advanced disease. The TNM breakdown is quite technical, but is provided here for your reference. 

AJCC TNM (2010) and FIGO tumor staging: Vulvar Cancer

Primary Tumor (T)

T

FIGO Stages

Definition

TX

 

Primary tumor cannot be assessed

T0

 

No evidence of primary tumor

T1s*

 

Carcinoma in situ (pre-invasive carcinoma)

T1a

A

Lesions 2 cm or less in size, confined to the vulva or perineum and with stromal invasion 1.0 mm or less**

T1B

IB

Lesions more than 2 cm in size or any size with stromal invasion more than 1.0 mm, confined to the perineum

T2***

II

Tumor of any size with extension to adjacent perineal structures (lower/distal 1/3 vagina, anal involvement

T3****

IVA

Tumor of any size with extension to any of the following: upper/proximal 2/3 of urethra, upper/proximal 2/3 vagina, bladder mucosa, rectal mucosa, or fix to pelvic bone.

* FIGO no longer includes Stage 0 (T1s)

** The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion.

*** FIGO uses the classification T2/T3. This is defined as T2 in TNM.

**** FIGO used the classification T4. This is defined as T3 in TNM.

Regional Lymph Nodes (N)

N

FIGO Stages

Definition

NX

 

Regional lymph nodes cannot be assessed

N0

 

No regional lymph node metastasis

N1

 

One or two regional lymph nodes with the following features

N1a

IIIA

1 or 2 lymph node metastases each 5mm or less

N1b

IIIA

One lymph node metastasis 5mm or greater

N2

IIIB

Regional lymph node metastasis with the following features

N2a

IIIB

Three or more lymph node metastases each less than 5 mm

N2b

IIIB

Two or more lymph node metastases 5 mm or greater

N2c

IIIC

Lymph node metastasis with extra-capsular spread

N3

IVA

Fixed or ulcerated regional lymph node metastasis

Distant Metastasis (M)

M

FIGO Stages

Definition

M0

 

No distant metastasis

M1

IVB

Distant metastasis (including pelvic lymph node metastasis)

Stage Groupings

Stage

T

N

M

0

T1s

N0

M0

I

T1

N0

M0

IA

T1a

N0

M0

IB

T1b

N0

M0

II

T2

N0

M0

IIIA

-IIIAi

-IIIAii

T1 or T2

T1 or T2

T1 or T2

N1a or n1b

N1a

N1b

M0

M0

M0

IIIB

-IIIBi

-IIIBii

T1 or T2

T1 or T2

T1 or T2

N2a or N2b

N2a

N2b

M0

M0

M0

IIIC

T1 or T2

N2c

M0

IVA

T1 or T2

T3

N3

Any N

M0

IVB

Any T

Any N

M1

What are the treatments for vulvar cancer?

Surgery, radiation therapy and chemotherapy are the typical treatment options. Depending on the cancer stage they may be used as a single treatment modality or in combination.  

Possible treatment options by stage are as follows:

Stage 0 (carcinoma in situ)

  • Left untreated will likely progress to invasive vulvar cancer.  Laser surgery, wide local excision or a skinning vulvectomy may also be treatment options depending on location and size of the cancer. 

Stage I

  • Radical local excision (a deep excision of the tumor) with or without removal of all nearby groin/upper thigh lymph nodes or sentinel lymph node biopsy.
  • Partial radical vulvectomy and removal of nearby groin lymph nodes (and sometimes lymph nodes on opposite side of the body) or sentinel lymph node biopsy.
  • Radiation therapy alone (in selected patients).

Stage II

  • Modified radical vulvectomy and removal of groin lymph nodes on both sides of the body or sentinel lymph node biopsy. Postoperative radiation therapy to the pelvis with or without chemotherapy if lymph nodes are positive for cancer.
  • Radiation therapy alone (in selected patients).

Stage III

  • Radical vulvectomy and removal of groin/upper thigh lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis and groin if lymph nodes are positive for cancer or if the primary vulvar tumor is very large.
  • Neoadjuvant chemoradiation (chemotherapy and radiation given prior to surgery) is often used in advanced cancers to shrink the tumor in order to minimize the surgery needed or eliminate the need for surgery altogether.
  • Radiation therapy (in selected patients) with or without chemotherapy.

Stage IV

  • Neoadjuvant chemoradiation (chemotherapy and radiation given prior to surgery) is often used in advanced cancers to shrink the tumor in order to minimize the surgery needed or eliminate the need for surgery altogether.
  • Pelvic exenteration is used rarely due to the extent of the surgery and the effect on quality of life. Exenteration entails radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread), as well as the uterus, cervix, and vagina.
  • Radiation therapy with chemotherapy, with or without surgery.

Clinical Trials

Clinical trials are extremely important in furthering our knowledge of this disease. It is through clinical trials that we know what we do today and many exciting therapies are currently being tested. Talk to your healthcare provider about participating in clinical trials in your area.

Recurrence of Vulvar Cancer

Local recurrence (near where the cancer was originally found) is the most common place where cancers can recur. These can often be treated with surgery. If the disease has spread to other organs (metastatic or distant disease), it may be treated with chemotherapy. The most frequently used chemotherapy regimens are called "platinum-based", meaning they consist of cisplatin, given alone or combined with another agent, such as 5-FU, paclitaxel, vinorelbine, or mitomycin C.

Side Effects of Vulvar Cancer & Cancer Treatment

Many of the side effects from surgery and radiation occur due to the close proximity of the bladder and rectum to the vulva. Due to this close proximity, these organs can be damaged during surgery or radiation. Side effects from the radiation can include irritation of the bowel and bladder resulting in diarrhea and increased frequency or urgency of bowel movements or urination. This typically resolves within a few weeks of finishing treatment, though it can become a long-term concern for some women.

Radiation can cause scar tissue to form in the vagina and the tissue can become dry and less elastic. There may be some shrinking of the vagina and vaginal opening. Scarring of the vaginal tissue can result in "adhesions", or areas where scar tissue forms, sealing the sides of the vagina together. This can make it difficult for a provider to perform vaginal exams and makes sexual intercourse difficult and uncomfortable. Your oncology team will teach you to use vaginal dilators to reduce the severity of this side effect. Rarely, a connection between the bladder or rectum and the vagina can form (also known as a fistula), which allows passage of stool or urine into the vagina.

Damage to the drainage (lymphatic) system in the area, by radiation or surgery to remove lymph nodes, can lead to a chronic swelling called lymphedema, which can occur at any time after treatment. Studies have found this condition to occur in anywhere between 14-48% of women who undergo lymph node dissection for vulvar cancer. Notify your healthcare provider if you develop any swelling in the legs or pelvis. A survivor with lymphedema who develops pain or redness in the leg(s), especially with fever, should be evaluated right away, as these signs may indicate infection. Some women may be good candidates for sentinel lymph node biopsy (SLNB), which appears to greatly reduce the risk of developing lymphedema. In SLNB, only the lymph nodes that the tumor area drains to are removed, limiting the damage done to the lymph system. SLNB is performed by gynecologic oncologists that have been trained in this procedure.

Follow up care and survivorship

After treatment for vulvar cancer, you will be followed closely by your healthcare team.  In general, is it recommend that you have a pelvic exam every 3-6 months for the first 2 years after treatment, then every 6-12 months for years 3-5 after treatment and then annually based on risk for recurrence. Other tests including cervical/vaginal cytology screenings, radiology, CT or PET scans and lab tests may also be performed depending on symptoms or suspicions of recurrence. 

More than half of the women who undergo vulvectomy report sexual dysfunction and psychological issues. The extreme changes in a woman's anatomy can result in painful intercourse, body image concerns, decreased desire, inability to orgasm, and difficulty with urination. Adequately preparing a woman for the expected changes, providing tools for coping with these changes, and discussing the emotions surrounding the cancer and its treatment may help some women. Women should not hesitate to talk openly with their healthcare providers about their feelings and fears and consider professional counseling to help in healing emotionally, as well as physically.

Fear of recurrence, the financial impact of treatment, employment issues and coping strategies are other common emotional and practical challenges experienced by vulvar cancer survivors. Your healthcare team can identify resources for support and management of these challenges faced during and after cancer.

Cancer survivorship is a relatively new focus of oncology care. With some 15 million cancer survivors in the US alone, there is a need to help patients transition from active treatment to survivorship. What happens next, how do you get back to normal, what should you know and do to live healthy going forward? A survivorship care plan can be a first step in educating yourself about navigating life after cancer and helping you communicate knowledgeably with your healthcare providers. Create a survivorship care plan today on OncoLink.

Resources for more information

Eyes On The Prize: information and emotional support for those affected by gynecologic cancers. Has a helpful discussion board where you can "chat" with other women.

Society of Gynecologic Oncology: Professional organization of gynecologic oncologists. Find a specialist tool.

The Labia Library: An Australian based website that answers many common questions about the labia.

References

American Cancer Society, Vulvar Cancer, www.cancer.org/cancer/vulvar/detailedguide

National Comprehensive Cancer Network (NCCN) Guidelines: Vulvar Cancer (2016). (log in required) https://www.nccn.org/professionals/physician_gls/f_guidelines.asp

Alkatout I, Schubert M, Garbrecht N, Weigel MT, Jonat W, Mundhenke C, Günther V. Vulvar cancer: epidemiology, clinical presentation, and management options. Int J Womens Health. 2015 Mar 20;7:305-13.

Aragona AM, Cuneo NA, Soderini AH, Alcoba EB. An analysis of reported independent prognostic factors for survival in squamous cell carcinoma of the vulva: is tumor size significance being underrated? Gynecologic Oncology. Mar 2014; 132(3):643-648.

Barlow EL, Hacker NF, Hussain R, Parmenter G. Sexuality and body image following treatment for early‐stage vulvar cancer: a qualitative study. Journal of Advanced Nursing. 2014 Aug 1; 70(8):1856-66.

Graham K, Burton K. "Unresectable" vulval cancers: is neoadjuvant chemotherapy the way forward? Current oncology reports. Dec 2013; 15(6):573-580.

Han K, Louie AV, Marchand E, Leung EW, Milosevic M, Fyles A. What Is the Optimal Treatment for Patients With Sentinel Node–Positive Vulvar Cancer?  International Journal of Radiation Oncology- Biology-Physics. 2014 Sep 1; 90(1):S503. 

Kidd, E et al. ACR Appropriateness Criteria management of locoregionally advanced squamous cell carcinoma of the vulva. American Journal of Clinical Oncology. 36(4):415-22, 2013 Aug.

Reade CJ, Eiriksson LR, Mackay H. Systemic therapy in squamous cell carcinoma of the vulva: current status and future directions. Gynecologic Oncology. Mar 2014; 132(3):780-789.

Regauer S, Reich O. Etiology of vulvar cancer will impact on treatment options and therapy outcome: Two major pathways of vulvar cancer. Gynecologic Oncology. Oct 2013; 131(1):246-247.

Viswanathan C, Kirschner K, Truong M, Balachandran A, Devine C, Bhosale P. Multimodality imaging of vulvar cancer: staging, therapeutic response, and complications. AJR. American Journal of Roentgenology. Jun 2013; 200(6):1387-1400.

Wills A, Obermair A. A review of complications associated with the surgical treatment of vulvar cancer. Gynecologic Oncology. Nov 2013;131(2):467-479.


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