An overview of disease, diagnosis and treatment
The vulva is a woman’s external genitalia. It is composed of the skin, connective tissue and fatty tissue surrounding the clitoris and the openings of the vagina and urethra. Cancers often occur in, or on, labia majora or labia minora, and more seldom on the clitoris, or in the Bartholin’s glands on the sides of the vaginal entrance. Vulva cancer is named from the cell type, where the cancer starts. The most common type is a squamous cell carcinoma, which originates from the cells in the outer layer of the skin in vulva. Other less common types are adenocarcinoma (from glandular tissue), malignant melanoma, and sarcoma. The treatment options for vulvar malignancy are dependent on the tumor type, size, location and possible spread to lymph nodes and/or distant sites in the body.
Epidemiology
The disease mostly affects older women (over 70 years), but it can also be diagnosed in younger women. The cancer may be solitary or multifocal. As the location of the disease implies, the disease and the treatment may cause visible sequelae following treatment.
Symptoms
Vulva cancer can present in various ways, incl. as a red scaling and/or itching flat area, as either a protruding or deep growing tumor, or as a not healing wound.
In early stages, vulva cancer rarely has any symptoms. As the disease progresses, the cancer may develop symptoms incl. palpable swelling/tumor in the area, itching, burning, pain (e.g. during urination), and bleeding.
Reporting any symptoms early to your primary physician is important for detecting vulva cancer in early stage, where the malignancy may be curable.
Causes And Risk Factors
The exact cause of vulva cancer is not known, but several conditions predispose (are risk factors for) developing vulvar malignancy.
- Infection with Human Papilloma Virus (HPV)
- HIV infection
- The autoimmune disease Lichen sclerosis
- Smoking
- Family history of malignant melanoma
Diagnosis
Squamous cell carcinoma is the most common histological type of vaginal cancer. It develops in the squamous cells in the mucous membrane, which lines the internal surface of the vagina. If not treated, the carcinoma may grow deeper into the vaginal wall, and eventually penetrate the vagina and invade e.g. bladder or rectal tissue nearby. The cancer can also spread to distant sites incl. bones, lungs, and liver.
Vulva Cancer Types
Squamous Cell Carcinoma
This is the most common histological type of vulva cancer. It develops in the squamous cells in the skin lining the vulva. If not treated, it may spread superficially or grow deeper into nearby tissue incl. anal region and urethra. The cancer can spread to lymph nodes and distant sites incl. bones, lungs, and liver.
Melanoma
Malignant Melanoma may be diagnosed on the skin in the vulva region. Treatment for this type of cancer is different from the treatment described below for the squamous cell carcinoma in vulva.
Examinations
Biopsy
A biopsy is a small tissue sample from suspicious areas/tumor. Often several biopsies are taken to thoroughly investigate a tumor and surrounding tissue. Biopsies are send for histological examination by a pathologist, and are warranted to make a definite diagnosis of cancer incl. the type.
If a biopsy shows invasive vulva cancer, the patient must be referred to a specialized gynaecologic oncologist department, where there is expertise in treating this type of cancer.
Colposcopy
Vulvar colposcopy is performed by specialist gynaecologists. The colposcopy is an instrument (microscope), which the doctor uses to inspect for abnormal tissue, tumors and bleeding in the vulva.
In case of irregular/suspicious area(s), the doctor will perform biopsies for histological examination.
Computed Tomography (CT) Scan
A CT scan visualizes the inside of the body using x-rays from different angles. Intravenous infusion of CT contrast fluid enhances the details on the CT-scan, and aids the radiologist in distinguishing normal from pathologic tissue.
On a CT scan the tumor’s size can be measured, and any enlarged lymhp nodes or pathologic tumors in other organs be shown.
Magnetic Resonance Imagin (MRI) Scan
A MRI scan uses magnetic fields to produce detailed images of the body. Often the tumor size is more precisely measured on a MRI scan, instead of on a CT or PET/CT scan. MRI is also valuable to detect invasion/growth in to neighboring organs.
Positron Emission Tomography (PET) or PET-CT Scan
A PET scan is usually combined with a CT scan (see above), and the combined scan is called a PET-CT scan. It can provide additional information regarding a cancer, than only a CT scan can do.
For a PET scan a small amount of a radioactive sugar substance is injected intravenously into the patient before the scan. The radioactive substance is taken up by the most energetic cells in the body, which includes cancerous cells.
A PET scanner then detects the distribution of this substance in the body, and by adding these data to a CT scan, the combined examination can with great details demonstrate size, location, extent and possible spread of the cancer.
This is very useful information for the doctor to have, before determining the optimal treatment strategi for a patient.
Endoscopy
An endoscopy allows the doctor to see inside the body via a thin, flexible tube with light in the end. Hereby the doctor can examine e.g. the bladder (cystoscope) or the rectum (rectoscope).
Treatment options
Radiation Therapy
Radiation therapy is an option for treating vulva cancer, and is used for irresectabel cases with or without spread to lymph nodes.
Surgery may be performed more than once on a patient, but if radicality is not obtainable, then radiation therapy is a better option, as it may be curable in selected cases.
It may also be given after surgery to reduce the risc of recurrence.
Radiation therapy uses high-powered energy beams (usually fotons) to destroy and kill cancer cells. The cancerous tissue (tumor) is irradiated from the outside by “external beam radiation therapy”.
During radiation treatment care is taken to protect the surrounding normal tissue such as bladder, and rectum etc. However, it is often unavoidable that a limited dose affects the surrounding tissue, and consequentially results in side effects of varying degree.
For vulva cancer these will often be redness, desquamation, and ulcers in vulva, pain, urinary problems, diarrhea, inflammation of the mucous membranes in the vagina, bladder, and rectum. Development of scar-tissue in the vulva area (fibrosis), and swelling af the leg(s) due to lymphoedema is common.
Discuss the risks and possible treatment options with your radiation oncologist prior to commencing your treatment.
Surgery
Surgery is the treatment of choice for early stage, small, and localized vulva cancer. The surgeon performs a local excision of the cancerous tissue with a margin of healthy tissue surrounding it. Lymph nodes may be removed also.
Vulvectomy is a more comprehensive surgical procedure, used for more advanced stages of vulva cancer. In a vulvectomy all or part of the vulva is resected.
A number of women who have undergone aggressive vulvectomy, may need reconstructive surgery(ies) incl. specialized plastic surgery with skin grafts performed by skilled surgeons.
Chemotherapy
Chemotherapy intravenously may be a treatment option for women with previously irradiated, recurrent or disseminated vulva cancer.
It can be prescribed together with radiation therapy to increase the effectiveness of the radiation.
Making Decisions About Your Care
The choice of therapy for your cancer is an individual decision, and many factors are taken in to consideration, when your doctor(s) discuss the best option(s) with you. These include the type, size and location/spread of the tumor, as well as your age and general health status.
We recommend, that you have a thorough discussion with your doctor(s) about the results upon your diagnosis, the treatment-plan and your chances of recovery (e.g. prognosis).
You are encouraged to include family and/or friends for participation in doctors appointments etc. for improving your/their understanding and remembrance of relevant information, as well as for emotional and physical support during appointments and treatments.
Your doctor can give a detailed explanation of the various therapy options, and has knowledge of the possibility to participate in a clinical trial. Each patient is different and so is their wish upon information needed/wanted prior to making important decisions.
Well designed and conducted clinical trials are essential to determine the effectiveness of a promising new drug or intervention. Many gynaecologic oncology departments have studies open for inclusion during the course of your cancer treatment. Talk to your doctor(s) about your thoughts regarding possible inclusion in a clinical trial now or in the future.
Author: Kristine Madsen, MD, Medical Director NSGO-CTU
