What is the Vulva?
The vulva is the external female genital organ composed of 3 portions- the "Labia Majora," "Labia Minora," and "Clitoris." The skin between the vagina and anus ("Perineum") is considered an extension of the vulva. The urethral opening for urination is close to the clitoris, in the upper vulvarregion. Thus, the vulva is the vaginal "lips" and surrounding area. A cancer of the vulva is not considered "vaginal" cancer, since it arises outside of the vagina proper and behaves differently, tending to spread to different areas. There is a system of draining channels ("lymph channels") to drain blood serum from the vulva. The drained blood serum is purified by glands ("lymph nodes"), which are normally pea-sized but swell ("lymphadenopathy") when invaded by infection or cancer. Specifically, the vulva usually drains first to lymph nodes in either upper thigh ("inguinal nodes"), it afterward may drain to lymph nodes in the pelvis proper ("pelvic nodes"). These pelvic nodes then interconnect (via lymph channels) to those in the abdomen ("paraaortic nodes"); the filtered blood serum is finally rejoins the bloodstream above the level of the heart (via the "left thoracic duct"). The lymph glands, which are normally filled with White Blood Cells, are important as they can act as a conduit for the spread of infections or cancer . Initially, at least, disease of the right half or the vulva spreads to the right inguinal nodes, and that of the left vulva to left inguinal nodes. A cancer is the midportion of the vulva spreads equally to both right and left inguinal nodes. There is some "interconnection" between the right and left lymph nodes, so this rule is not steadfast, but it is useful "clinically" to doctors. Furthermore, the vulva has a rich blood supply and venous drainage, which can also promote spread of disease to anywhere in the body. However, this distant spread tends to occur only long after the areas("regional") lymph nodes are involved. The vulva can be removed ("vulvectomy") which interferes with sexual function, but it is not considered a "vital organ" (necessary to live).
What is Vulvar Cancer?
The vulva is composed of various "cells," which are intricately combined together into "tissues" which form the "organ" . The vulva contains fat, muscle and skin cells. These cells divide to produce new ones, and grow very rapidly during womb life, early childhood and puberty. In adulthood, new cells are produced only to replace those that die of old age, injury or disease. Normally, division of cells is under very tight control. This control is exerted by the "genes" inside each cell, which are housed in long clumps forming "chromosomes," which are visible under a light microscope. The genes themselves are made up of DNA, the master genetic code material. If the genes are damaged, say by chemicals or radiation, the control over cell division may be lost in one particular cell. Ultimately, cancer is considered a disease of the DNA. Vulvar cancer starts in a single cell . That cell starts dividing haphazardly, making millions and billions of copies of itself. It takes up the nourishment needed by other cells, depriving them so the cancer can continue to grow. Quickly growing cells can clump up to form a "tumor" . A tumor simply means a swelling, it can be caused by inflammation or infection. A "benign" tumor only grows in it's local area (although it may get quite large)-- it cannot spread and is not cancer. By contrast, a tumor which can spread to other body areas is called "malignant" and this is cancer . The process of cancer spread to other areas is called "metastasis," so only malignant tumors (i.e. cancer) can metastasize. Theoretically, cancer can spread to any area of the body, and it often grows better in it's area of spread than in it's area of origin ("primary site") . It is this capacity for spread that makes cancer so dangerous. If not treated successfully, vulvar cancer ultimately kills by urinary blockage, debility, anemia, infection, and damage to distant organs like the liver and brain.
How Does Vulvar Cancer Spread?
The most common area for vulvar cancer to start is on the Labia Majora, it is three times more common here than on the Labia Minora. Only about 10% of cases initially involve the Clitoris. When a cell turns pre-cancerous, it may start dividing but stay localized for many years, or even many decades. This is called"Vulvar Intraepithelial Neoplasia ("VIN") . A portion of "VIN" cases progress to"Carcinoma in Situ" ("CIS"), which is technically the first stage of actual cancer. Only about 5% of VIN cases progress all the way to"Invasive Cancer," but we don't know in advance which ones will or won't. Once Invasive Cancer manifests, it tends to grow for month to years in it's local area. It then spreads to the Inguinal lymph nodes("lymphogenously"), usually to one side first. Then it spreads to deep pelvic lymph nodes. Only 4% of patients have pelvic lymph nodes involved("positive") in the absence of Inguinal lymph node involvement. The cancer may then track up through the lymph channels to invade Paraaortic lymph nodes in the abdomen. It continue to grow locally to invade the skin, urethra, bladder, perineum, rectum, and pelvic bone . It tends to spread through the bloodstream ("hematogenously") only late in the disease, mostly to the liver, lung, bone and brain.
How Common is Vulvar Cancer?
There are about 3200 new cases of invasive vulvar cancer each year in the U.S.A., it accounts for1200 deaths annually. Vulvar cancer represents 4% of the total cancers involving the female genital tract ("gynecologic malignancies"). Overall, gynecologic malignancies account for 13% of new cancer cases in American women. Patients with the precancerous VIN condition have are an average of 44 years old. Frank cancer (but not VIN) is rare before age 50, and the average patient is 61 years old. The overall incidence (number of new cases annually) of vulvar cancer is steady. Over 90% of cases are "Squamous Cell Carcinoma," which originates from the "epithelial" (skin and lining cells) of the vulva (the ones overlying the fat and muscle). About 7% of cases are"Melanoma," arising from"melanocyte" pigment cells; this has more predilection for spread in the bloodstream."Paget's Disease of the Vulva" is a pre-cancerous condition showing on the vulvar skin a red and velvety area, it has an underlying "invasive cancer" in about 2% of cases. Paget's is a "marker" for the development of another gynecological malignancy (e.g. cervix or uterine cancer) which eventually occurs in 25% of patients with it. "Bartholin's Gland Cancer" is an "adenocarcinoma" (arises from gland cells) seen exclusively in post-menopausal women (over about age 50) it makes up less than 1% of vulvar cancer cases. Other rare possibilities are "Sarcoma" (from the underlying muscle or fat), "Lymphoma" (from the immune cells in the vulva) or "Basal Carcinoma" (a skin cancer) The treatment for these follows that in other body areas where they are more common.
What Causes, or Increases the Risk for Vulvar Cancer?
As for any cancer, the exact reason why one woman gets vulvar cancer and another does not remains unknown. However, these "risk factors" are often present:
1) Vulvar Intraepithelial Neoplasia (VIN) or Carcinoma in Situ (CIS) may exist for several decades before manifesting as Invasive Cancer, which they only go on to less in less than 5% of patients.
2) Viruses of the Genital Tract -- Specifically Human Papillomavirus ("HPV") which causes genital warts ("condyloma acuminatum"). Around 5% of patients with vulvar cancer have genital warts. Also Herpes Simplex II is also associated with VIN and later Invasive Cancer. These viruses are sexually transmitted ("STD's") .
3) Nulliparity means never having had children, about 25% of American women are "nulliparous." This is a risk factor for breast, uterus, and ovarian cancer too. Menopause at an early age (i.e. the mid 40's) has a higher risk also.
4) Lower Socioeconomic Status means being poor, especially as a member of a generally poorer group (Blacks, Hispanics, Native Americans). Some feel that the higher incidence is explained by more promiscuity in these groups (more chance of getting a Sexually Transmitted Disease from multiple male partners). It is probable that the poor don't get medical attention until the disease is further advanced, and have other medical problems associated with this cancer.
5) Other Medical Problems associated with vulvar cancer include being obese, having high blood pressure, heart disease, diabetes, and kidney problems. All of these conditions are related to each other.
6) Occupation and Environmental Exposure -- Women who worked in the Laundry or Custodian (cleaning) Industries have a greater risk, reason unknown.
What are the Symptoms of Vulvar Cancer?
Very early vulvar cancer has no symptoms, as it is too small to cause problems.
1) A Lump or Bump ("Mass") on the vulva is the most common first sign, it can become a sore ("lesion") which will not heal and grows slowly over months.
2) Itching ("Pruritis") and Pain will occur as the cancer grows in it's local area.
3) Bleeding will occur as the cancer breaks through the skin. It may be scant.
4) Lymph Gland Swelling ("lymphadenopathy") in the pelvis; this does not prove that the glands are involved since infection will also swell them. On the other hand, non-swollen glands may still be microscopically involved with cancer.
5) Urinary and Bowel Problems can occur as the cancer invades the urethra, bladder and rectum respectively.
6) Signs of Distant Spread include back pain (from spread to para-aortic lymph nodes), bone pain from spread there, or nervous system problems from brain spread. Vulvar cancer tends to grow large in it's local area prior to spreading.
***20% of patients have no previous symptoms when their disease is detected.
Is Vulvar Cancer Preventable?
There is no sure way of preventing vulvar cancer . Being careful to avoid getting a Sexually Transmitted Disease will lower the risk, as will good vulvar hygiene. If a woman has the risk factors for this disease, she should be especially vigilant about doing vulvar"self-exam" on a monthly basis to look for any new suspicious areas, and bring them to a doctor's attention without delay.
How is Vulvar Cancer Diagnosed and Evaluated?
As for any cancer, the only way to absolutely diagnose vulvar cancer is to get a sample of it ("biopsy") to examine under a microscope. Biopsy material is studied by a "pathologist," a physician specializing in diagnosing disease from tissue samples. If a patient comes ("presents") with suspicion of vulvar cancer, we do the following:
1) Complete History and Physical examination, the doctor notes"symptoms" (that which the patient feels, e.g. pain) and"signs" (that which can be measured e.g. tumor size or fever). A completepelvic exam is done with a Papaniculao ("PAP") smear of the cervix to look for cancer there. The groin is examined for enlarged glands ("inguinal nodes") and the size is noted if present.
2) Blood and Urine Tests are to assess general health; there are no special blood tests("tumor markers") yet to detect spread of squamous vulvar cancer as for some other cancers. Routine tests will include Complete Blood Count ("CBC") to look for anemia and infection. A Blood Chemistry Panel ("SMA") measures sodium, potassium, blood sugar, cholesterol and liver and kidney function. If a major surgery is contemplated, blood tests for clotting ability (PT, PTT and bleeding time ) are standard. A Urinalysis(UA) to check for protein, blood or infection completes the lab tests. A carefully collected ("clean catch") urine is sent for "culture and sensitivity" to identify the bacteria in it and the antibiotics that bacteria is susceptible to.
3) Imaging Tests are done in the radiology department and standardly include a Chest X-ray to look for signs of infection or lung tumors. An Intra-Venous Pyelogram ("IVP") if often used to see if there is any obstruction blocking the kidneys, ureters, or bladder. Recall that advancing vulvar cancer often will invade these areas. IVP is also good for identifying radio-opaque kidney stones, a common cause of blood in the urine and pelvic pain. IVP is generally a safe test; the main danger is an allergic reaction to the injected dye, or the kidneys shutting down from this dye . When cancer invades the ureter, anus, rectum or bladder wall, tests are done for local spread. CT scan in "thin slices" of the tumor area helps define the extent of the tumor, and is excellent for looking for spread to lymph nodes in the pelvis which can not be detected on standard physical exam. This will also show swollen lymph nodes in the inguinal area. Another valuable test to study soft tissues and lymph nodes in the pelvis is the expensive magnetic Resonance Imaging(MRI) scan. MRI uses magnetism instead of radiation, allows the area to be viewed in three dimensions. It is great for looking at the tumor as well as checking local muscle, fat and nerves for signs of invasion by cancer. Contrast material can be injected into the blood-stream prior to either CT scan or MRI, the contrast runs through and highlights blood vessels. If getting a CT with contrast, ask for "omnipaque" contrast; other types are less expensive but more likely to cause an allergic reaction. For MRI scans, a different type of contrast, called "gadolinium" is used. If bowel invasion is suspected, a Barium Enema may be ordered, where some contrast is put through the anus and X-rays taken. Other more exotic tests are only obtained in the presence of suspicious symptoms. For instance, a bone scan is gotten if there is new bone pain, a CT of the chest is ordered when the plain Chest X-ray appears to show tumor in the lung, and a CT of the Brain is gotten if new neurological symptoms occur. If major surgery is contemplated, Lung Function Tests ("FEV") may be done in the pulmonology department to assess lung capacity. An EKG is gotten to rule-out recent heart attack, and if heart abnormalities are suspected astress-thallium and/or echocardiogram look at the heart. Tests can be ordered to look at any area of the body - but only if necessary. There is no point in ordering a possibly painful and expensive test if it's results cannot change the planned therapy for the patient.
4) Cystoscopy and Proctoscopy means putting a visualization tube up the urethra and into the bladder, and up through the anus and into the rectum, respectively. These tests are done by a "gynecological oncologist," who specializes in cancers of the female genital tract, or a "urologist," who specializes in general urinary tract cancers. We need to see if the inner lining ("mucosa") of the bowel and/or bladder is invaded, as this will have treatment consequences. These tests may be done during a deep pelvic "Exam Under Anesthesia" (EUA).
5) Biopsy (sampling) of the tumormakes the ultimate diagnosis of whether cancer is present or not, and specifies the particular type. A biopsy may be "excisional" for a small tumor, meaning the whole thing is removed with a surrounding safety margin, or "incisional" for a large tumor, taking a small piece to confirm or deny cancer. Normally a "wedge biopsy" is taken of vulvar sores ("lesions") which will not remove the whole suspicious area unless it is very small (less than 2 cm~ about 1 inch). This biopsy is taken under local anesthesia with numbing lidocaine. The biopsy material will be sent to the pathologist who examines it with special stains, looking for the frequent cell divisions and abnormal nuclei (center of the cells) that signifies cancer. The pathologist will often "grade" the cancer by how aggressive it looks."Grade I" looks a lot like the normal tissue or origin and doesn't have very many cell divisions, it tends to be non-aggressive ("indolent")."Grade III" has very many cell divisions, scarcely resembles the tissue of origin, and tends to be very aggressive."Grade II" is intermediate in behavior. If melanoma is seen, the pathologist specifies the "depth of invasion" into the skin ("Clark's or Breslow"), which is important for determining whether melanoma has spread distantly. A pathologist's report often takes several days (of anxious waiting) to complete.
How is the Extent of Vulvar Cancer Gauged?
As for all cancers, the extent of vulvar cancer is gauged by the"Stage" . The most commonly used staging system is the Federation of Gynecology and Obstetrics ("FIGO") system which uses Roman numerals I- IV for advancing cancer:
Stage I means the cancer is smaller than 2 cm (~ 1 inch) and confined to to the vulva or surrounding skin, with no lymph nodes involved .
Stage II means the cancer is larger than 2 cm and still confined to the vulva or surrounding skin, with no lymph nodes involved .
Stage III means the cancer has invaded theurethra, and/or anus, and/or with "unilateral" (one side of the groin) lymph node involvement .
Stage IVA means the cancer invades thedeeper urethra, bladder, rectum, or bone, and/or "bilateral" (both sides of the groin)lymph nodes involved .
Stage IVB means any distant spread ("metastasis"), includingpelvic lymph nodes, liver, lung, distant bone or brain. .
What is the Survival with Vulvar Cancer?
This depends upon the subtype of cancer, stage, therapy selected and other medical conditions the patient suffers from("comorbid conditions") . In general:
Stage I 95% (survival at 5 years)
Stage II 80% "" " ""
Stage III 60% "" " ""
Stage IVA 35% "" " ""
For stage IVB, with pelvic node involvement, survival drops to about 20%, and with more distant spread including para-aortic lymph node involvement, to about 10%. However, note that some patients with para-aortic lymph node involvement are still curable. With distant spread to lung, liver, bone or brain survival falls to less than 5% at 5 years.
***The above textbook numbers are AVERAGES, they do not tell how well any given patient will do. The numbers include death from all causes, including heart attack, accident, or some other cancer. Remember that many patients with vulvar cancer are elderly and have other serious "comorbid" medical conditions. Also note that many patients live high quality lives, often for years, with "incurable" cancer, if the symptoms are properly managed("palliated") .
What Determines Whether Vulvar Cancer is Curable?
Factors that favor or disfavor cure are called "prognostic factors," and are learned by observing large series of patients to see how well they do. For vulvar cancer:
1) Unfavorable Factors include more advanced stage, location involving the bladder or rectum, lymph nodes involved, and ability to tolerate therapy. If one lymph nodes is involved, survival at 5 years may be 95%, if two nodes are involved, survival drops to 80%, and with three or more involved, 15%.
2) Favorable Factors include Low Grade, Low Stage, lack of lymph node involvement (especially in the pelvis) and ability to tolerate aggressive therapy.
What is the Conventional Treatment for Vulvar Cancer?
The Conventional Treatments for vulvar cancer have been Radical Surgery for those who could tolerate it, and Radiation Therapy for those who were not "surgical candidates." Each of these is now described in detail:
Surgery has been, and remains, the mainstay of treatment for vulvar cancer, and the treatment to which other methods are compared for effectiveness. Surgery is ideally performed by a "Gynecologic Oncologist" a Board-Certified specialty in cancers of the female genital tract. For early cancers of the vulva, the surgery is a straightforward "local excision" which may even be done under local anesthesia at the time of biopsy. If the goal is to remove the entire lesion, it must be accompanied by an appropriate "safety margin" to account for cells that have migrated from the main tumor. When the biopsy specimen is examined by the pathologist (s)he will state whether"clear margins" were obtained-- that is no tumor cells are seen at the edge of the specimen. This is obviously very important if local excision is to be the only therapy, for if "clear margins" were not obtained, we can assume that residual cancer was left behind . The patient will then need to undergo another surgery for "re-excision" to obtain clear margins. A main problem with other methods of destroying early tumors, such as burning ("fulguration"), freezing with nitrogen ("cryotherapy") or electrically destroying them ("electrodessication") is that there is no pathological specimen to send to determine whether all the cancer was removed ! Interestingly, even if some cells were left behind, this does not automatically mean that the tumor will regrow, but it is very possible. Thus, "wedge biopsy" which removes the entire lesion ("excisional biopsy") is the best choice for early vulvar cancers.
Very early cancers, or Carcinoma in Situ ("CIS") has almost zero chance of having spread to local lymph nodes in the groin ("inguinal nodes") and so these nodes are not sampled unless they are enlarged. However, as a cancer grows, the chance of spread to local lymph nodes increases proportionately. If the cancer is not removable with simple excisional biopsy, then a more radical procedure is needed, and the local lymph nodes will be sampled. If the cancer is on just one side of the vulva, the appropriate surgery is "hemivulvectomy with ipsilateral inguinal lymph node dissection" which means a major surgery under general (or at least spinal) anesthesia. One-half of the vulva is surgically removed, and the open wound covered with a skin flap from the thigh or buttock. An incision is made into the groin on the same side as the vulvar lesion, and several lymph nodes are removed (with particular attention to any enlarged ones). The materials are sent to a pathologist during the operation, to make sure a "clear margin" is obtained on the vulvar tumor, this is done quickly by a technique called "frozen section." More detailed information will have to await the special stains, and thin serial slicing of lymph nodes, that the pathologist will do later. Recovery from a hemivulvectomy takes about 3 days of hospitalization, and the tissues heal to 75% of their normal strength in 3 weeks (so heavy lifting is again possible). Risks include a 2% chance of operative death (many vulvar cancer patients have other complicating medical conditions), 10% risk of infection, and 5% chance of the surgical wound splitting open ("dehiscence") . The operation is curative for most stage I and II cancers, but close follow up is essential. If clear margins were not obtainable, or if lymph nodes were involved, the patient is standardly recommended for post-operative (after surgery) radiation treatments.
If the cancer involves the midline, or both halves of the vulva, the only curative surgery will be a "total vulvectomy with bilateral inguinal lymph node dissection." This is significantly more radical than the above hemi-vulvectomy, with about double the complication risks quoted above. As the name implies, both sides of the vulva are removed under general anesthesia, with a skin graft to cover the surgical wound. A separate incision is usually made over each upper thigh (inguinal) area, and the local lymph nodes sampled on each side. Healing time is generally longer with a total vulvectomy. In the past, if the inguinal nodes were "positive" (had cancer), the pelvic lymph nodes would also be surgically removed ("pelvic lymphadenectomy") . If the inguinal lymph nodes were not involved, there was only about a 3% chance of the pelvic lymph nodes being involved, so it that case this procedure was omitted. Doing such radical pelvic surgery had consequences of poor sexual function in most patients, and possible lower extremity swelling ("edema") from lymphatic disruption.
The most radical surgery was reserved for when the bladder or rectum were involved with cancer (stage IVA). In this case, surgical cure might be achieved with an "exenteration" procedure. Exenteration basically means removing most, or all, of the pelvic organs (which would be coupled with a total vulvectomy and inguinal lymph node dissection for vulvar cancer). An "anterior exenteration" removes the cervix, uterus, ovaries, and bladder, as well as pelvic lymph nodes. A "posterior exenteration" removes the cervix, uterus, ovaries, and rectum. A "total exenteration" removes all of these organs and might be used if both the bladder and rectum were invaded by cancer. Obviously, this is an extreme operation, and it has about a 10% death risk, 20% infection risk, and 15% risk of suture breakage ("dehiscence") somewhere in the pelvis. There is also a significant chance of lower limb edema. If the rectum is removed, a"colostomy" is required, where the colon is brought out ('"externalized") to the abdominal wall, and stool collected in a disposable bag. If the bladder is removed, the draining ureters from the kidneys can be externalized to a disposable "cystostomy" bag on the abdomen to collect urine. Alternately, a false bladder can be formed ("Koch Pouch") or the ureters implanted into the rectum (if it remains) to drain urine. The side-effects and complications of exenteration make it only a procedure of the last resort to try and save the patient's life ; much of it's success in fighting locally advanced cancer can be duplicated, or improved upon, with less dangerous and less debilitating "radical radiation therapy" . The exenteration is often reserved for cases where radical radiation therapy has failed, and no good option is left to save the patient's life-- in this case it may be 25% or so curative.
Radiation Therapy has been, as still is, used to treat patients who are not surgical candidates. This may be because of co-existing medical problems, such as recent heart attack or stroke, or because the cancer is too far advanced to be removed by a reasonable surgery. Radiation therapy was very seldom used for patients in whom surgery could be done; the results were somewhat poorer for radiation than surgery -- of course, sicker patients and those with greater disease were being radiated . Thus, the effectiveness of radiation was not (until recently) as appreciated as it should have been. Also, there have been many technical improvements in radiation therapy which have enhanced it's effectiveness. Like surgery, radiation therapy is a local treatment, but a wider"field" may be radiated than removed surgically. As will be seen, it can be combined with other therapies to increase "local control" of vulvar cancer , since return of vulvar area disease has been a major problem for unsuccessfully treated patients. Radiation Therapy can also help relieve ("palliate") the symptoms caused by distant spread of the disease. Treatment is administered under a "Radiation Oncologist," a cancer doctor who specializes in utilizing radiation.
Radiation kills cancer cells by damaging their DNA, they die when they try to divide. Thus, damaged cancer cells die even after the treatment is complete. Radiation will also kill normal cells, which limits the amount that can be given.
However, it usually takes more radiation to kill normal cells than cancer cells, and normal cells can often repair the radiation damage, while cancer cells can not. Nevertheless, it is important to be as exacting as possible in the administration and dose of radiation, so as to minimize the injury to adjacent normal cells. Particular areas of concern when radiating the vulva include the rectum and small bowel and bladder .
To receive therapy, a patient is first seen in"consult" by a radiation oncologist, who reviews the patient's medical record, complaints, and radiology films. After explaining the possible benefits and side-effects of radiation, the patient is scheduled for a "simulation." This means the area to be treated is marked out on a replica treatment machine, and films are taken. Some barium contrast material is usually squirted up the patient's rectum. Watercolor marks are painted on the patient to denote the treatment area, and eventually small, permanent tattoos are placed on the skin. Sometimes the patient is sent for a CT scan along with the simulation, the whole process takes less than 2 hours, and is painless. Information from the simulation and relevant scans is placed into a "treatment planning computer," which generates a"plan." This plan tells how much radiation is going to the tumor area, and how much to adjoining normal tissues. For vulvar cancer, particular attention is paid for how much radiation is going to the bladder, rectum, and small intestine. Often, the beam is aimed from 4 directions (front, back, right and left sides) to uniformly dose the bladder. Higher energy treatment machines (over 15 Megavolts) also help smooth out the dose to the bladder. The plan is reviewed by the radiation oncologist and also by a specially licensed Radiation Physicist prior to starting therapy. The patient then comes in for their "treatment start." They are placed on a hard, flat table in a specially shielded room and aligned with laser lights.
The actual treatments are given by "Radiation Therapists," or "R.T.T's," who are first certified for diagnostic X-rays and then get additional training to deliver therapy. For the first treatment, "verification films" are taken to ensure proper positioning; they do not tell anything about the cancer. The actual treatment only takes a couple of minutes and is given with a Linear Accelerator (or occasionally older Cobalt-60) which precisely aims a beam of photons at the treatment area. The head of the machine can swivel about the patient, to give the treatment from different angles. The patient needs only to lie still. Areas that are not to be treated can be "blocked" with special lead-type blocks in the head of the treatment machine. Normally, patients area treated 5 days a week, Monday through Friday, taking only several minutes each day. The usual dose for vulvar cancer is 70 Gray (units of radiation) given at 10 Gray per week. If a treatment is missed, it is simply tacked on to the end so full prescribed dose it given. It is common to "cone down" off of the full pelvis after 50 Gray (since this is the tolerance dose for the small bowel) and shrink the field to treat the vulva proper only. This is called a "boost." Be aware that treatment normally covers the major lymph nodes in the pelvis. To cover the inguinal area properly, a pad material ("bolus") is often used to cover the groin;will boost up the dose to the shallow tissues, important for full inguinal node coverage. Alternately, the inguinals can be "boosted" up with a electron beam field, which is less penetrating than the photons used to treat deeper tissues.
Radiation to the pelvis area is painless, the patient does not become "radioactive", nauseated or lose their scalp hair . The patient can usually maintain normal activities, such as working, driving, and intake of alcoholic beverages. The side-effects of External Beam treatments are classified as "acute" (during treatment) or "late" (months to years after treatment). The most common acute symptoms are reddening of the skin in the treatment area, and anal area irritation. After several weeks of radiation therapy, it is common to develop frequent urination and diarrhea as the bladder and rectum (respectively) become irritated. Prescription of soothing steroid suppositories and anti-diarrheal medicine is usually all that's necessary to treat these acute effects; some dietary modification (less fruit and fiber) may also help. There is often a sense of greater fatigue while receiving radiation. As the dose "builds up" with successive treatments, more blood in the urine may be seen. In general, however, radiation treatments are very well tolerated, the expected side-effects are confined to the treatment area, and abate after completion of therapy. Of more concern are possible "late" effects, which tend to be long lasting or permanent if they occur. Specifically, impotence develops in about 50% of irradiated patients, bladder constriction ultimately requiring cystectomy in 10%, chronic diarrhea in 5%, and bowel or urethral obstruction requiring eventual surgery in 5%. Incontinence is rare (2%) as are second cancers caused by radiation (<1%). Giving the treatment as many fractions, instead of in one large dose, helps reduce the incidence of late reactions. The patient returns for follow-up after completion of treatments, seeing both their gynecologic oncologist and radiation oncologist. If all goes well, they are ultimately seen once or twice yearly for routine check. Overall, radiation treatments are safer than surgery. The results of radiation therapy alone for invasive vulvar show about 60% of patients surviving 5 years-- which is the same as surgery! This is especially remarkable since most of these patients have more advanced tumors or are medically sicker than surgical patients.
Radiation therapy may be used to shrink stage III and IVA tumors, to make them amenable to surgical removal. When surgery has been performed first, and two or more inguinal nodes are involved, post-operative radiation therapy reduces local recurrence and increases long-term survival . In this case the dose given is lower, 50 Gray, since we are only radiating for "microscopic residual" disease, all the "gross tumor" having been removed surgically. Also patients with clitoral tumors are usually treated first with radiation therapy, since surgical removal of the clitoris will reduce sexual pleasure and is hard to psychologically accept by the patient. It is common to have problems with vaginal dryness as a Late Effect, since radiation damages the glands which lubricate the vagina. Topical estrogen creams are helpful at alleviating this condition, called "atrophic vaginitis." For patients with stage IVA tumors that would require surgical exenteration, radical radiation gives about a 30% cure rate, with less complications than surgery. Moreover, if radiation does fail, then exenteration can be considered for "salvage" and works about 25% of the time. In general, while the Acute Effects of radical radiation may be hard to take, with a lot of anal, vaginal and perineal irritation, the outcome is worth the temporary discomfort. CancerAnswers has available an In-Depth Transcript about Radiation Therapy which may be ordered through our Web Site .
Chemotherapy alone has not been curative for vulvar cancer . Active agents include 5-Fluorouracil, Cisplatinum, Methotrexate, Mitomycin-C and Bleomycin . The "response" to these agents is only about 20%, with very few patients having a "complete response" (total disappearance of tumor). Also, responses tend to be short-lived, lasting an average of only 3 months . Thus, intravenous chemotherapy alone has not been heavily utilized for vulvar cancer patients. It has mostly been used in an attempt to "salvage" patients with recurrence after surgery or radiation therapy. However, it may be much more effective when combined with radiation treatment and/or surgery, called"multi-modality" therapy. We will examine this option in "Latest Effective Treatments" . CancerAnswers has available an In-Depth Transcript about Chemotherapy which may be ordered through our Web Site .
What is the Latest Effective Treatment for Vulvar Cancer?
The best treatment for early lesions, including VIN, CIS, and Stage I invasive cancer with "less than 1 millimeter deep invasion" is local excision with close follow-up to make sure the lesion does not recur. A"radical" local excision is employed, so that the pathologist can determine whether the requisite"clear margins" are present. If not, the lesion will need to be re-excised. Burning or Freezing the lesion is discouraged, except perhaps for the earliest "warty type" ("verrucous") VIN, very close follow up is mandatory if this avenue is taken. For Paget's disease of the vulva, there may be an underlying adenocarcinoma in a couple percent of patients, so it is insufficient just to remove the red-velvety lesion. The underlying dermis (full thickness skin) should also be sent for pathologic evaluation to obviate ("rule-out") deeper cancers. For patients who seem to have a tendency to develop early vulvar cancers, it is important that the full pelvic exam is done routinely (i.e. semi-annually) with Pap smear done, for these patients are at increased risk to develop cancers in other areas of the female genital tract (e.g. vagina, cervix, uterus & ovaries).
A relatively new treatment, AFTER APPROPRIATE EXCISIONAL SURGERY, is the use of Topical (spread on the skin) 5% 5-Fluorouracil ("5-FU") cream . This is usually spread over the vulva on seven consecutive days, with 3 treatment "cycles" being at least 2 weeks apart. This "topical chemotherapy" vastly reduces the number of new vulvar cancers forming, and treats residual cancer cells that were missed at surgery. It may irritate the area, but otherwise has no appreciable body wide ("systemic") side effects, as it is only local treatment. Studies show it about 90% effective at preventing recurrence, and the treatment may be repeated if necessary. This therapy is only effective for squamous cell cancer or precancer, not for melanoma of the vulva.
For patients with deeper invasion of vulvar cancer, up to about 5 millimeters into the skin, surgery remains the first choice (except for Clitoris cancer or patients who can't tolerate surgery). A "radical local excision with ipsilateral (i.e. same side)inguinal lymph node dissection " is done for lesions on one size of the vulva ("lateralized") -- see"Conventional Therapy" for explanation of this surgery. If the cancer is in the mid-portion of the vulva, then both "groins" (inguinal areas) are have lymph nodes removed, usually via separate incisions by the gynecologic oncologist. If positive lymph nodes are found in the groin, it is appropriate to add extra ("adjuvant") radiation therapy to 50 Gray over 5 weeks to the groins and pelvis, both since pelvic lymph nodes may be involved, and to reduce the recurrence risk in the groin. For Clitoris cancer or patients who can't tolerate surgery, "primary radiation therapy" with at least 65 Gray should be given to the cancer area (including the groin), but the pelvic dose should be limited to 50 Gray to minimize Late Effects there. This 50 Gray is sufficient for mopping up"microscopic disease", but higher doses (i.e. 65 - 70 Gray in 7 weeks) are necessary for "gross disease" . Since we cannot re-irradiate to these high levels, it is crucial to do it right the first time with an expert radiation doctor.
For patients with stage II or greater ("locally advanced") vulvar cancer have been treated with either hemivulvectomy or total vulvectomy, with bilateral inguinal lymph node dissection. Patients who had massive local disease got"exenteration" procedures (See section on Conventional Therapy). Using separate incisions to remove groin lymph nodes reduces the risk of lower extremity swelling ("edema") and wound breakdown("dehiscence") . The latest effective treatment for these patients is to use"combination pre-operative chemo-radiation" to shrink the tumor, followed by radical local excision of any residual tumor and "bilateral groin lymph node dissection." This"combination therapy" is the same strategy as has been used successfully for anal and esophagus cancers, it is available at University Academic Medical Centers. It should be administered jointly by cooperation between the gynecologic oncologist and radiation oncologist . Insist upon proper specialists, and not "general surgeons" or "medical oncologists" to the surgery and chemotherapy. The chemotherapy agents commonly used for combined treatment are Intravenous 5-Fluorouracil, Mitomycin-C, and Adriamycin . These agents can cause lowering of blood counts, intense local irritation, nausea and diarrhea, and baldness and heart damage (Adriamycin). CancerAnswers offers a much more In-Depth understanding of Chemotherapy is available through our Web Site. Cisplatin, which is very useful in other gynecologic tumors, appears to have little activity against vulvar cancer, and has side effects including nerve damage, kidney damage and hearing loss. Better results may be gotten with 5-Fluorouracil, the most popular agent, if it is given slowly by"infusion pump" instead of in large injected doses ("bolus") . The results of chemo-radiation show: For locally advanced vulvar cancer, over 60% of patients appear cured in limited series, and 50% of "salvage patients" (treated after failed surgery) stay free of disease for long periods (years) .
A common place for recurrence after surgery, especially in patients who did not receive radiation, is the groin. This can lead to severe open wound problems with cancer spreading on the skin, infection and pain. Radiation treatment with Hyperthermia means heating the area (to about 110 degrees F.) and then delivering radiation. If External Beam with electrons or photons has already been given to the area, an"implant" of radioactive material("brachytherapy") over several days may control the tumor, with minimal dose to surrounding normal tissues. Also 5-Fluorouracil cream and Retinoic Acid cream (a vitamin "A' derivative) can be spread over the area to fight existing cancer and minimize new cancer formation.
Treatment for Melanoma is discussed separately in a specialized transcript.
When vulvar cancer is stage IVB, with distant spread through the body, the objective is no longer cure but symptom relief ("palliation"). The patient should always be made as comfortable as possible, and narcotic medicines like morphine (which Osler called "G-d's own medicine) should never be withheld for fear of causing "addiction." Using "Fentanyl Patches" applied to the skin helps give a continuous amount of narcotic, eliminating the problems of forgotten doses, "loss" of narcotics, and smoothing out the dosing for less disturbing "highs and lows." Importantly, Radiation Treatment can help pelvic pain, urinary obstruction, bleeding and bone pain in over 80% of patients. It is also useful for reducing the symptoms, and even extending survival, in patients with spread to the brain. Sometimes radiation therapy is used as an emergency measure when the cancer spreads to the spinal column and threatens to cause paralysis by pressing upon the spinal cord. Any patient with bladder cancer who experiences new weakness of the extremities, numbness, or loss of bowel or bladder function must be brought into the Emergency Room immediately to see whether the tumor is compressing the spinal cord causing these symptoms . Up to 60% of new back pain in a cancer patient is caused by spread of cancer there. The patient is given a painless Magnetic Resonance Imaging (MRI) scan to check for "epidural spinal cord compression" . If this is caught early, and treatment is given, permanent paralysis may be prevented. It is unfortunately uncommon to reverse symptoms of paralysis once they have set it, however, so quick recognition is essential.
As mentioned,radiation treatment can be very helpful for metastatic vulvar cancer. A relatively new method of radiation for spread to the brain (one of the most common areas of spread) is "Stereotactic Radiosurgery", where multiple beams of convergent radiation are aimed onto the area(s) of spread in brain, in a single painless session of one afternoon. This is usually followed by 10 to 20 treatments with conventional "External Beam" radiation. The advantage of Stereotactic Radiosurgery is that it can give a very high dose of radiation to areas of brain metastasis, and possibly enhance survival for these patients, without the risk of an open brain surgery from a neurosurgeon.
Other options for patients in severe pain for multiple areas of spread to bone include "hemi-body" radiation, and "strontium-89." Hemi-Body radiation uses a low dose (6 to 8 Gray) in a single treatment to the upper or lower body to treat multiple areas of bony involvement; some anti-nauseants are usually necessary and it lowers blood counts. It is over 90% effective for pain relief lasting an average of 6 months. Strontium-89 is an injected radioisotope that goes through the bloodstream to all bony areas, and is especially attracted to cancerous areas. It also lowers blood counts but is very effective at palliating pain. It can only be done once. If no relief is gotten from medications or radiation, neurosurgical techniques to cut sensory nerves can usually afford relief, to this small population of patients. Committing suicide because of unrelieved pain should NEVER be necessary with pain science today.
The patient with newly diagnosed vulvar cancer should not rely on any one therapy, but instead should use a combination approach to maximize the chance for success Specifically, besides the conventional medical therapies mentioned above, consider the use of a non-toxic, not over-expensive alternative therapy that you believe in, a program of spiritual renewal, "mind over cancer', nutritional therapy and exercise. Keep the most positive attitude possible-- research has shown this to be an important factor in survival. CancerAnswers has a transcript available on reasonable alternative treatments which you can order through our web-site . Using a true"multi-modality" approach will give the confidence that you have done everything possible for a happy outcome, and certainly improve the current quality of life.
In conclusion, new techniques utilizing surgery, radiation therapy and chemotherapy are showing better survival rates than ever before for vulvar cancer. If cure is the objective, consider getting treatments at a University Academic Center and joining ongoing research trials which offer the latest therapies. The National Cancer Institute keeps a listing of open cancer trials on their website (they change frequently). Especially look for ones listed as "Intergroup" studies, or in conjunction with the Radiation Oncology Therapy Group ("RTOG") . The future has never looked brighter for vulvar cancer patients!
This is the full transcript, offered freely in the spirit of internet sharing, of CancerAnswers' report on Vulvar Cancer. Much more, including latest
additional treatments for Vulvar Cancer can be found on our order page. Thank you for using CancerAnswers as
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ADDITIONAL TOPICS
Acute Leukemia
Anal Cancer
Bladder Cancer
Bone Cancer
Brain Cancer
Breast Cancer: Early
Breast Cancer: Advanced
Cartilage Cancer
Cervical Cancer
Chronic Leukemia
Colo-rectal Cancer
Esophagus Cancer
Fat Cancer
Gall-Bladder Cancer
Hodgkin's disease
Kidney Cancer
Larynx Cancer
Liver Cancer
Lung Cancer
Lung "small cell" Cancer
Lymphoma
Melanoma
Mesothelioma
Mouth Cancer
Multiple Myeloma
Muscle Cancer
Muscle and Fat Tumors
Nasal Cavity Cancer
Nasopharynx Cancer
Ovarian Cancer
Pancreas Cancer
Penile Cancer
Plasmacytomia
Prostate Cancer
Skin Cancer
Stomach Cancer
Testicle Cancer
Thyroid Cancer
Tongue Base and Tonsil
Cancer of Unknown Origin
Uterine Cancer
Vaginal Cancer
Vulvar Cancer
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