William M. Rich, MD

This is a term that includes several conditions that are associated with the results of a pregnancy. The conditions are molar pregnancy, invasive mole, metastatic mole and gestational choriocarcinoma(korio carcinoma). These are cancers and cancer like conditions of placental elements. The concept is so far beyond most people's experience, that unless they have been to medical school they will never have heard of it. It is not uncommon.

The easiest way to explain this disease is to start at the beginning. In the beginning, a single egg produced by the mother is fertilized by a single sperm from the father. The egg and the sperm are unique because each has only one chromosome from each of the 23 pairs of chromosomes found in all other cells. At fertilization the complete complement of 23 pairs is thus restored. The fertilized egg divides and forms two daughter cells. Before division occurs it must duplicate its chromosomes so that each daughter cell has a complete complement of 23 pairs. This type of cell division is called mitosis. These two daughter cells divide by mitosis and so there are now four identical cells each with the normal 23 pairs of chromosomes. Further cell division results in 8, then 16, then 32, then 64 identical cells. Then things start to become complicated.

Certain cells begin to differentiate and become different from the other cells. Some of the cells will eventually form the extra-embryonic tissues, that is, the placenta, the membranes (bag of water), and the umbilical cord. The fetus is eventually formed by the remaining cells. The placenta is composed of three elements. The villi, the cytotrophoblast cells and the synctiotrophoblast (sin shishio tro fo blast) cells. The villi, or villus when describing only one, is a microscopic finger like structure containing a fetal blood vessel. It invades into the lining of the uterus. The synctio- and cytotrophoblast cells surround the villi and help the villi to erode into the maternal blood vessels in the wall of the uterus.

There are millions of villi in a placenta. Oxygen and nutrients that are supplied to the fetus from the mother's blood must traverse the villus to be picked up by the fetal blood vessel in the villus. There is one fetal blood capillary per villus. The fetal blood is separate from the maternal blood. The villi and the cyto- and synctiotrophoblasts have to invade the lining of the uterus to reach the maternal blood vessels. As the pregnancy progresses the number of villi initially increases then begins to decrease as the placenta ages. At birth the placenta separates and along with the membranes and umbilical cord is discarded. They have done their job. This is the normal way the placenta functions. The invasion into the lining of the uterus is similar to the invasion of a cancer, but in pregnancy this is normal.

Sometimes something goes wrong very early in pregnancy. The fetus does not develop but the placental elements continue to grow. There is swelling of the villi and overgrowth of the cyto-and synctiotrophoblast cells. The villi can become so swollen that they are visible and look like drops of water. The scientific name for this mass of water drops is hydatidiform mole. In Latin mole means shapeless mass and hydatid means water drop. It is referred to as a mole or molar pregnancy. The trophoblastic cells make the pregnancy hormone, Human Chorionic Gonadotropin, HCG, which is the basis for all pregnancy tests. There is an overproduction of HCG as well as exaggerated symptoms of pregnancy.

Eventually, the patient will spontaneously miscarry and pass the mole. If the molar pregnancy is detected before that happens then an abortion has to be done to evacuate the uterus. Obstetricians are well familiar with this condition and can diagnose it by a sonogram. There is a characteristic appearance to the uterine contents, and there are no fetal structures or heart beat. Only very rarely will there may be a coexisting fetus.

Why molar pregnancies occur is unknown, but there are some remarkable features about them:

They have the ability to invade into the wall of the uterus.

They can develop into choriocarcinoma which is a virulent cancer.

They have 23 pairs of chromosomes, all of which are paternal in origin.

They are XX, and both of the X chromosomes are also of paternal origin.

The incidence in Asia is about 1 in 120 pregnancies.

The incidence in Northern Europe is 1 in 2000 pregnancies.

In the USA the incidence is about 1 in 1500 pregnancies.

Metastatic disease sometimes undergoes spontaneous regression.

After the molar pregnancy is evacuated there must be rigorous surveillance for any sequelae. The consequences of a mole can be persistent mole, invasive mole, metastatic mole or choriocarcinoma. The follow up is done by a weekly blood test for HCG. Actually, it is for a specific sub-unit of the HCG molecule called B-HCG (Beta HCG). The B-HCG may be in the millions and has to fall to less than 2. Usually the blood test is normal within 8 weeks. Then it is repeated every month for 6 months and then every other month for 6 months. During this time the woman should not become pregnant again because that will also increase the B-HCG, and make things complicated.

If the B-HCG decreases but then levels off and starts to rise again, then the diagnosis is Gestational Trophoblastic Disease. This may be either invasive mole (mole growing into the wall of the uterus), metastatic mole, usually to the lungs, or choriocarcinoma. At this point the patient is reexamined, a chest x-ray obtained and perhaps a scan of the liver. But for sure, the patient needs chemotherapy. This is one case where chemotherapy is given on the basis of a blood test without a tissue diagnosis. If there is B-HCG, and the patient is not pregnant, she must be treated.

Treatment is usually easy. A single chemotherapeutic agent is given and repeated every two weeks until one course of treatment is given after the titer is normal (titer is the level of B-HCG in the blood). Then the patient is followed for a year with monthly B-HCG titers. As long as they remain normal everything is normal. After the year is up the patient can become pregnant again. The risk for another molar pregnancy is about doubled. But that is still a small number. If it were 1 in 1500 for the first mole it would be 1 in 750 for the next pregnancy.

Molar pregnancies and their management is the easy part. The problem is when they are ignored, not followed adequately, or inadequately treated, because then major problems occur. If a previous pregnancy ended in a miscarriage and there was no pathologic specimen it may have been an unknown molar pregnancy. If the last pregnancy was a normal term pregnancy and delivery, then nobody would be expecting choriocarcinoma to develop. But it can and it is usually not diagnosed promptly. It can be anywhere in the body and is a very aggressive cancer. It metastasizes widely and early. It is very invasive and destroys the tissue. It bleeds profusely. If it is in the brain then signs of a stroke or seizure may occur; if in the lung then the patient may cough up blood; if in the uterus then irregular bleeding. A simple pregnancy test that is positive will indicate the diagnosis.

Gestational trophoblastic disease is characterized as either metastatic or nonmetastatic. If nonmetastatic then treatment is by single agent chemotherapy or sometimes by hysterectomy. If metastatic, then it is divided into good prognosis and poor prognosis disease.

Poor prognostic disease indicates the need for more aggressive chemotherapy. This means a combination of drugs or the addition of surgery and or radiation to the treatment plan. The major concern is that it be treated aggressively.

There is also a World Health Organization Scoring System to define the good and poor prognostic groups.

The scoring systems indicate the need for multi-agent chemotherapy. The high risk groups and the poor prognosis group requires aggressive multi-drug regimens. Resistant areas that can be irradiated are irradiated. Involved organs or parts of organs that can be removed are removed surgically, because if the B-HCG titer does not go down to normal and stay there, the patient is going to die.

These patients should be treated at a center experienced in treating this disease. They will need extensive therapy and support. The treatment is vigorous and at times ruthless. This is a cancer that can be cured, even though widely metastatic. The prognosis depends on the extent of disease and the aggressiveness of treatment. If a molar pregnancy is managed properly, the cure rate is about 100%. If non metastatic trophoblastic disease is vigorously treated the cure rate is also about 100%. Widely metastatic disease if recognized promptly and treated aggressively with multi-agent chemotherapy, surgery and radiation if necessary, is curable in about 80% of the cases.


 Clinical Professor of Obstetrics and Gynecology
 University of California, San Francisco
 Director of Gynecologic Oncology
 University Medical Center
 Fresno, California

Articles

Gestational Trophoblastic Disease

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 Cancer of the vagina is rare. It is almost always a 

 that occurs in women who were exposed to DES (diethylstilbestrol) in-utero. One of the reasons that it is rare is that cancers of the vagina that also involve the vulva are considered to be vulvar cancers; if it involves the cervix it is considered to be a cervical cancer. Vaginal cancer may cause symptoms of abnormal bleeding and foul discharge. Bleeding after intercourse is a symptom of cancer of the vagina as well as cancer of the cervix.

There is a premalignant phase for squamous cell cancer of the vagina similar to the squamous cell cancers of the vulva and cervix. The premalignant phase is vaginal 

 grade III (VaIN III). This is also sometimes called carcinoma-in-situ. The premalignant phase is usually asymptomatic but can be detected by routine Pap test. It can be treated by excision, laser evaporation or occasionally by a chemotherapy type of vaginal cream. There is no recognized cause for vaginal 

 or cancer, although it is similar to the squamous dysplasias of the cervix.

Invasive squamous cell cancer of the vagina is usually treated by radiation. Although it can be removed surgically, the bladder or rectum or both would have to be removed with it in order to get a good margin around the cancer. As a general rule squamous cell cancers of the vagina do not spread early, so they are usually localized to the pelvic area on diagnosis. This is a good situation for radiation since that area can easily be irradiated and radioactive material can easily be placed into the vagina next to the cancer. The prognosis for localized disease is good.

Adenocarcinomas of the vagina associated with DES exposure were more frequent in the 1970's and 1980's. DES, diethylstilbestrol, is a synthetic estrogen hormone that was given to pregnant women in the 1950's to try to prevent miscarriages. It was not effective and its use discontinued. The female infants of these women, who took the DES, had some developmental abnormalities of their vaginas and cervices that put them at risk for developing a particular type of adenocarcinoma called a clear cell carcinoma. This particular problem has mostly run itself out as time has elapsed. There is some suggestion that they may be at risk for squamous cell cancer of the vagina or cervix and possibly even at an increased risk for breast cancer. The person who has studied this condition the most is:

Arthur L. Herbst, M.D.
 Chairman, Department of Obstetrics and Gynecology.
 University of Chicago School of Medicine.
 5841 S. Maryland.
 Chicago, IL 60637.

Women who have had a hysterectomy for non cancer problems should still have a Pap test every several years. They can still develop malignant and pre-malignant vaginal changes.

Cancer of the vagina is rare. It is almost always a squamous cell cancer. The exception is an adenocarcinoma that occurs in women who were exposed to DES (diethylstilbestrol) in-utero. One of the reasons that it is rare is that cancers of the vagina that also involve the vulva are considered to be vulvar cancers; if it involves the cervix it is considered to be a cervical cancer. Vaginal cancer may cause symptoms of abnormal bleeding and foul discharge. Bleeding after intercourse is a symptom of cancer of the vagina as well as cancer of the cervix.

Cancer of the Vagina

terms will open definitions in new windows

The cervix is the part of the uterus connected to the upper vagina. It is 			the structure that dilates during childbirth to allow the baby to traverse the birth canal. There are two major 			types of cancer that develop from the cervix. 

 that covers the visible part of the cervix.           

  			lining of the endocervical canal. About 85% of cervical cancers are squamous cell cancers and the remainder adenocarcinomas. 			Each of these major types has several subtypes that may require special treatment; otherwise they are all managed 			similarly. Squamous cell cancers are unique because there is a well established progression through premalignant 			changes before a cancer develops. These premalignant changes are easy to detect by a simple screening test called 			the Pap test.

The cause of cervical cancer is unknown. There is a strong association 			with certain subtypes of the Human Papilloma Virus (HPV) for the squamous cell cancers. HPV can be transmitted 			sexually, so there is an association with sexual activity. The strongest association, however, is that women who 			have been celibate all their lives almost never develop a squamous cell cancer of the cervix. They are at risk, 			however for an adenocarcinoma of the endocervix. Infection with HPV, which also causes genital warts, is common; 			cervical cancer is not. HPV changes are often noted on the Pap test report and should not cause alarm. If there 			are any 

 changes diagnosed then 			they will be treated. Treatment of these premalignant changes is usually simple and almost 100% effective.
 
 Sixty years ago cancer of the cervix was the leading cause of cancer deaths in women in this country, surpassing 			even those from breast cancer. The death rate began to fall in the 1940's and has continued to fall, in spite of 			the sexual revolution, third world immigration and the prevalence of HPV. The reason for this large decrease is 			unknown. It began before Pap test screening became prevalent. Cervical cancer is still the leading cause of cancer 			deaths in women in many third world countries. There are about 16,000 new cases diagnosed each year in the USA, 			with about 5,000 deaths. There are about 180,000 new cases of breast cancer each year and about 60,000 women die 			of lung cancer each year.
 
 All sexually active women are at risk for the development of cervical cancer. The risk seems to be increased with 			smoking and promiscuity of the woman or her male partner. This is the standard text book explanation for an increased 			risk. But, in my experience, it is not a likely explanation for the patients that I have treated. It is not increased 			by the use of birth control pills, family history or the development of genital warts. Almost all cases occur in 			women who have not had regular screening with Pap tests. This is one cancer that can be prevented, in most cases, 			by screening for the premalignant changes.

Screening means to test for the presence of a cancer before 			there are any symptoms or findings on examination. If there are symptoms or abnormal findings on examination then 			a diagnostic test must be done; not a screening test. The major benefit of the Pap test is to detect changes on 			the cervix before they become cancerous. These 

. 			They are easily and effectively treated.
 
 When a Pap test is reported as abnormal a well established evaluation is begun. Only after this evaluation is completed 			can a diagnosis be made as to the true condition of the cervix. Only after the diagnosis is established can treatment 			be recommended. It is a major mistake to treat on the basis of an abnormal Pap test without a diagnosis. An abnormal 			Pap test is not a diagnosis. It is only an abnormal screening test that must be evaluated. This evaluation is described 			in 

There may be no symptoms of a very early cervical cancer, 			but by the time it is large enough to detect visually it is usually symptomatic with abnormal bleeding. Often this 			abnormal bleeding occurs after sexual intercourse. Cancers must make new blood vessels as they grow. These new 			blood vessels are often abnormal and break easily which is why bleeding is a sign of cancer. The cancer also outgrows 			some of its blood supply, so portions of it are deficient in oxygen. This causes some of the cells to die and for 			the tissue to become infected. In the cervix this causes a foul discharge that will be noticeable and resistant 			to most treatments for the usual vaginal infections.
 
 As the cancer increases in size it usually grows laterally toward the pelvic wall. The tubes from the kidneys (ureters) 			that bring urine to the bladder pass through this area and they are easily obstructed. If that happens to both 			of the ureters, then this will result in renal failure, coma and death. If the cancer grows into the pelvic wall 			it will press on the nerves that go to the leg and cause unremitting leg pain. These are symptoms of an advanced 			cancer. Premalignant changes have no symptoms and are usually not noticeable on visual examination.
 
 Cervical cancers usually do not spread early. They tend to be slow growing and cause most of their problems in 			the pelvis. Although distant 

  			occur they are usually late events. Cervical cancers can spread by way of the           

. The lymphatic vessels drain from the cervix to clusters of lymph glands along 			the pelvic wall. The lymphatics follow the large blood vessels so the route of drainage is upward along the pelvic 			wall, then along the midline of the backbone and then to the chest. If the pelvic lymph nodes on one side of the 			pelvis become obstructed with cancer then that will cause swelling in the leg on that side. This is another sign 			of advanced cancer.

The diagnosis of cervical cancer is usually not difficult. 			It is usually big enough to be seen and can be biopsied. If it arises from up inside the cervical canal then it 			may not be visible. This will require that a portion of the cervix be removed for diagnosis. These large biopsies 			can be accomplished by either a 

 procedure. A major mistake is 			to rely on a Pap test to rule out a cancer in a woman who has symptoms or findings that could be due to a cancer. 			A normal Pap test never excludes a cancer. Cancer can only be excluded by the proper biopsies. It is known that 			about 10% of women with an obvious cancer of the cervix will have a Pap test that is essentially normal. This is 			because there is so much inflammation and dead cell debris that it masks the cancer cells. Very rarely, the cervix 			may be too small or inaccessible to biopsy properly. In these situations a simple hysterectomy may have to be done 			for diagnosis.

Whenever a cancer is diagnosed the next step is staging. 			This is a determination of the extent of the cancer. For cervical cancer this is determined by physical examination, 			chest x-ray, kidney x-rays and looking inside the bladder and rectum. CT scans and MRI scans can be done but they 			are not used to assign a stage. Likewise, surgical exploration is not used to assign a clinical stage.

In general, cancers of the cervix are treated with radiation. 			The major exceptions are for those that are stage I and some that are stage IV. Stage IA cancers that invade less 			than 3mm deep can sometimes be treated by simple hysterectomy or even in special cases by cone biopsy. All other 			Stage I cancers are treated either by radical surgery or radical radiation. Some stage IIA cancers can also be 			considered for surgery. Otherwise, all stage II, III and IV cancers are treated with radiation. Occasionally ultra-radical 			surgery is done on some stage IVA cancers. Surgery for stage IB and some IIA cancers requires a radical hysterectomy 			and removal of the pelvic lymph nodes. Radical hysterectomy means that the cervix is removed by staying as far 			away from it and the cancer as possible. A regular or simple hysterectomy removes the cervix by staying as close 			to it as possible.
 
 Cancer surgery requires that the cancer be removed with as good a margin of uninvolved tissue as can safely be 			taken. The radical hysterectomy technique removes all the supporting ligaments to the cervix which means that the 			dissection is very close to the bladder and to the rectum. The ureters have to be dissected out and the tissue 			around them removed. A radical hysterectomy with removal of the lymph nodes takes about 4 hours to perform. A simple 			hysterectomy takes only about 1-2 hours. The ovaries are not a part of the problem with cervical cancer and can 			be left in place. If after surgery the pathology indicates that there are positive lymph nodes or that the surgical 			margins are close, then pelvic irradiation with or without chemotherapy may be advised.
 
 Ultraradical pelvic surgery for advanced or recurrent cancer means that all the pelvic organs are removed. The 			uterus and cervix, vagina, bladder and rectum are removed. Sometimes a vagina can be reconstructed. If the rectum 			can be reattached then there will be no need for a colostomy. Sometimes a continent urinary reservoir can be constructed. 			Otherwise a bag will have to be placed for the urine to drain through an ostomy in the abdominal wall. This ultra-radical 			surgery is done if there is an extensive cancer involving the bladder or rectum, but without spread beyond these 			structures. It is also done for cancers that recur after pelvic radiation if they are confined to the pelvis.
 
 Radiation therapy usually requires a treatment each day, five days a week, for about five weeks. Each treatment 			takes only several minutes. This is called external or teletherapy. The entire pelvic area is irradiated by an 			x-ray beam usually generated by a linear accelerator. Everything in the pelvis is irradiated, bladder, rectum, 			large intestine, small intestine, bone and skin. Following this treatment, a radioactive source is placed inside 			the cervix and vagina and left in place several hours or several days. This is called an implant, radium implant, 			intracavitary implant or any of several other names. A more accurate term is brachytherapy which means slow therapy.
 
 Often when cancer of the cervix is being treated with radiation, chemotherapy is also given to increase the effects 			of the radiation. Otherwise, chemotherapy is not used as initial treatment for cancer of the cervix. There are 			some investigational studies in which chemotherapy is given first and then either surgery or radiation performed.
 
 Complications from treatment with surgery are related to anesthesia and injury to other organs such as the bladder 			and ureters. There is also the risk associated with blood transfusions and infection. These complications usually 			occur early and are remediable. Radiation complications can occur years later and are difficult to fix.

Most early cancers are cured; most advanced cancers are not. 			If a cancer was removed surgically then it cannot come back. If it recurs that means that a cancer cell had already 			spread by the time the cancer was removed, and it took a couple of years to grow large enough to be detected. If 			a cervical cancer is destined to recur, about 85% will recur within the first two years after treatment. If there 			has been no recurrence by five years, then the cancer is unlikely to recur and is considered cured.

These are somewhat misleading numbers. Certainly, almost all stage IA 			cancers will be cured. Stage IB cancers, if the nodes are negative and the surgical margins adequate will also 			almost all be cured.

If the cancer recurs then the outlook is generally poor. 			If initially operated then the recurrence can be treated with radiation. If initially irradiated then sometimes 			ultra-radical surgery can still be curative. If there is a recurrence in the cervix or vagina after the pelvis 			has been irradiated and there is no evidence of cancer anywhere else, then ultra-radical surgery can be done. This 			is called a total pelvic exenteration. The uterus, tubes, ovaries, bladder, colon and vagina are removed. Sometimes 			the vagina can be reconstructed and the colon reattached. A continent urinary reservoir can also be done.
             
             Isolated recurrences elsewhere such as lung or liver can be removed surgically or irradiated if not removable. 			The treatment of isolated recurrences can be curative since cervical cancers do not usually spread widely throughout 			the body. Chemotherapy for recurrent cancer has not been very effective, but is often tried.

Cervical Cancer During Pregnancy 			
             

When an abnormal Pap test is obtained on a woman who is pregnant 			the evaluation is modified. In general, the pregnancy has no effect on the cervical problem and the cervical problem 			has no effect on the pregnancy. However, the cervix is best not manipulated or biopsied during the first trimester 			because the risk a spontaneous miscarriage is about 20%. Should this happen, the biopsy will be blamed, although 			it will not have been the cause.
             
             There is no urgency to diagnose a premalignant condition during pregnancy. All that is really necessary is to exclude 			or diagnose an invasive cancer. Often this can be accomplished by a colposcopic examination, without the need for 			any biopsy. The premalignant conditions can easily wait until 6 weeks after the baby is born to evaluate and treat. 			Sometimes however, biopsies and even cone biopsies must be done. The best time for these biopsies is the early 			second trimester because the risk for a spontaneous miscarriage has past and cervical manipulation during the third 			trimester risks premature labor.
             
             If an invasive cancer is diagnosed during pregnancy, the treatment is the same as for those not pregnant. Except, 			treatment can be delayed until the baby is sufficiently mature to be delivered if the diagnosis is made after the 			24th week of pregnancy. The baby will usually reach lung maturity sometime between the 32nd and 36th week.
             
             The best plan is to stay up to date with Pap test screening and prevent the development of this cancer.


             Clinical Professor of Obstetrics and Gynecology
             University of California, San Francisco
             Director of Gynecologic Oncology
             University Medical Center
             Fresno, California

The cervix is the part of the uterus connected to the upper vagina. It is the structure that dilates during childbirth to allow the baby to traverse the birth canal. There are two major types of cancer that develop from the cervix. Squamous cell cancers arise from the squamous epithelium that covers the visible part of the cervix. Adenocarcinomas arise from the glandular lining of the endocervical canal.

Cancer of the Cervix


 
 Cancer of the vulva is not a common disease. There are about 4,000 new cases each year in the United States. Although it can occur in women in the third and fourth decade it is usually diagnosed in older women. Over 95% of vulvar cancers arise from the 

. The remainder are mostly melanomas. The cause of squamous cancer of the vulva is unknown but there is a weak association with Human Papilloma Virus (HPV). The most important feature about vulvar cancers is the premalignant phase.

The premalignant phase of vulvar squamous cell cancer has several different names: carcinoma-in-situ, vulvar intraepithelial neoplasia grade III, (VIN III), severe dysplasia and Bowen's disease. This condition is diagnosed by tissue biopsy and is characterized by a full thickness disorder of maturation of the squamous epithelium (see 

). It is usually symptomatic with itching and burning and can be present for years. It is usually misdiagnosed as a yeast infection and a multitude of anti-fungal agents will have been prescribed, none of which will have been effective. It is easy to see on examination and will appear as a raised red, white or pigmented patch. A simple biopsy will confirm the diagnosis.

It is best treated by excision or sometimes by laser evaporation. If a large area is involved and must be removed, then a skin graft can be applied. These premalignant conditions are likely to recur after treatment so continued follow up is a necessity. Another condition that can occur on the vulva and also cause itching and soreness is called lichen sclerosis. It is not a premalignant change, but an atrophy of the skin. It will not be improved by anti-yeast medications either. It can be diagnosed by biopsy. These two conditions, lichen sclerosis and VIN III, can be present for years and be misdiagnosed as yeast infections.

The most important point about premalignant vulvar changes is that there is usually a long delay in diagnosis. Often these women are not examined properly or the examiner is unfamiliar with this condition and prescribes yet another course of cream, salve, or ointment. Usually the condition is fully visible and simply needs to be biopsied to establish the diagnosis.

Squamous cell cancer of the vulva usually causes pain, soreness and itching. There is usually an obvious growth on the skin or an ulcerated area. Diagnosis is by simple biopsy. These cancers are usually slow growing and do not spread early. When they do spread it is usually by way of the 

. The regional lymph nodes are located at the top of the thigh in the groin area. Vulvar cancers are staged by a combination of examination and surgery. The TNM staging system is used.

T-1A a cancer less than 2.0cm in diameter and less than 1.0mm in depth 			of invasion
 			T-1B a cancer less than 2.0cm in diameter but greater than 1.0mm in invasion

T-2 greater than 2.0 centimeters in diameter

T-4 involves bladder, rectum or pelvic bone

N-0 no lymph nodes involved
 			N-1 lymph node metastases to one groin
 			N-2 lymph node metastases to both groins

M-0 no distant metastases
 			M-1 any distant metastases

Vulvar cancers are usually treated by surgery with a radical excision of the cancer and removal of the regional lymph nodes. If the cancer is clearly on only one side and small then only that one side may need to be removed. Radical excision means that there must be a good margin of uninvolved tissue removed with the cancer. Usually an acceptable margin is about two centimeters. This will result in some disfigurement if the cancer is larger than about two centimeters in size. Large cancers will also require some sort of plastic surgery technique to close the defect. Complications of surgery are closure breakdown with prolonged healing and sometimes a collection of fluid in the groin where the lymph nodes where removed. There may also be leg swelling.

If the cancer is very large and a radical resection would require removal of the anus, rectum or urethra then primary treatment can be given by radiation to preserve these vital structures. If there is cancer in the lymph nodes then that groin as well as the pelvic lymph nodes are irradiated upon recovery from surgery. Often when these cancers are being irradiated chemotherapy will also be given to increase the effects of the radiation.

The prognosis is in general good. If the lymph nodes are negative then the chance for a cure is excellent. Even with positive lymph nodes a significant number are cured.

Vulvar melanoma is no different from melanomas that occur elsewhere on the body. They are unpredictable and can be very aggressive. They are treated surgically if possible. The regional lymph nodes are usually removed at surgery. Melanomas are characteristically black in color, however there are amelanotic melanomas that are not pigmented and can be confused with the usual squamous cell cancer.

Cancer of the vulva is not a common disease. There are about 4,000 new cases each year in the United States. Although it can occur in women in the third and fourth decade it is usually diagnosed in older women. Over 95% of vulvar cancers arise from the squamous epithelium. The remainder are mostly melanomas. The cause of squamous cancer of the vulva is unknown but there is a weak association with Human Papilloma Virus (HPV). The most important feature about vulvar cancers is the premalignant phase.

Author | William M. Rich, MD

Articles

Gestational Trophoblastic Disease

Cancer of the Vagina

Cancer of the Cervix

Cancer of the Vulva