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Vulvar Cancer

Vulvar cancer is relatively rare, accounting for about 3 to 5% of all gynecologic malignancies. It is the fourth most common malignancy of the female genital tract, and the incidence as high as the 7th decade of life and does increase with increasing age. The exact etiology of vulvar cancer, weíre not completely sure, there is a very high association with the high risk HPV serotypes, specifically type 16 and 18. Other associated factors are any chronic inflammatory condition, herpes has been implicated, obesity, diabetes, hypertension, prior squamous cell carcinoma of the cervix, vagina or the anal rectal area as well, and then vulvar dystrophy probably increases a womanís risk.

One of the main problems with this particular condition is that if affects older women, they often have other associated vulvar dystrophies, they may even have vulvar intraepithelial neoplasia, they often times are incontinent, they complain to their family physician, and there is often a delay of 2 to16 months between the onset of symptoms and the initial presentation to the physician. With the patientís being older and just denying that they have a particular problem. Then often times and in one series that was published out of MD Anderson, there was a delay because of medical treatment for up to 12 months or longer before definitive biopsy is performed. Symptoms include chronic pruritus, a lump or mass, pain, bleeding ulceration, dysuria and leg edema.

Intraepithelial lesions are treated a number of different ways, it depends on the site, the age of the patient and the size of the lesion. It is proper to excise with a 3 to 4 mm margin and then primarily close the area, this works well on the hair bearing areas and also for unifocal lesions. Sometimes if there is extensive perineal or perianal disease, or even periclitoral disease that encompasses a third to have of the vulva, sometimes you need to do a skinning valvectomy and place a split thickness skin graft, C02 laser and some people have used a Lupic to excise in the non-hair bearing areas, C02 laser I think is much better.

In order to make a diagnosis you need to get tissue, and wedge biopsy, excisional biopsies, colposcopy, itís very important to remember that you have to examine the remainder of the genital tract looking for vaginal lesions and also for cervical dysplasia or early invasive cancer because often times these can be metastatic from another site, and for patientís that have invasive carcinoma, we often times will get an abdominal pelvic CT scan to check for metastatic lesions in the pelvis or distant nodal areas. This just reminds me that doing a biopsy in the office is very easy, any lesion that is suspicious should be performed under local anesthesia, you want to make sure that if you have multiple areas, biopsy each one, record it on a drawing.

Squamous cell carcinoma is the most common followed by melanomas, about just under 6% of the time, Bartholin gland cancers are third, basal cell carcinoma, you see some sarcomas, you can very rarely see invasive Pagetís disease. The most frequent sites are on the labia majus, followed by the labium minorum, and then some patientís will have combined lesions about 15%.

For staging, and this is in your handout, staging was changed in 1995. For years we tried to identify if there was truly such a lesion as a microinvasive carcinoma of the vulva, similar to microinvasive carcinoma of the cervix, and several different institutions looked at trying to delineate risk of nodal involvement based on depth at 3 mm, 5 mm was where it was originally looked at...and there were several papers that had come out of MD Anderson suggesting that patientís who had less than 5 mm deep, also had a very similar low rate of nodal involvement similar to cervical cancer. When several of those patientís ended up recurrent, we found out that demarcation of 5 mm is probably not correct. It is more proper and correct, be believe that the demarcation for micro invasion is actually 1 mm or less. So stage I tumors, less than 2 cm in size with negative lymph nodes.

Stage IV disease is spread to the upper urethral bladder, rectal mucosa, pelvic bone and rectal mucosa is important for test question purposes, it cannot just be bullous edema, pelvic bone, or have bilateral regional lymph node involvement and stage IVB is a metastases or pelvic lymph node metastases.

The surgical treatment for vulvar cancer has changed quite a bit over the past two to three decades, back in the early 1900s Basset from France who adopted a Hallstedian concept to the treatment of vulvar cancer very similar what Dr. Hallstead had adopted for breast cancer, felt that wide surgical excision was the best, however, back in the early days, surgical technology was poorly developed, it didnít have antibiotics, they didnít have the capability to do blood transfusion and many of these women had huge tumors and then five years survival although 20 to 25% was felt to be encouraging very similar to advanced breast cancers.

The standard treatment, when I was a fellow in training and as a resident, was N block radical vulvectomy with bilateral inguinal femoral lymphadenectomies and we did selective pelvic lymphadenectomies through separate extra peritoneal incisions and this basically is what has been called the butterfly incision or the Texas longhorn incision.

The rationale for conservative surgery is that most of the metastases occur by embolization and the early advocates of the more conservative procedures in their series found no metastatic lesions in the skin bridge between the vulva and the groin, so you didnít need to remove that piece of tissue. There was also relative increase in T1 tumors in recent series, about 50% of lesions are much smaller than in the historical controls. Local recurrence rates are about 3 to 10% and most of those can be cured with reexcision locally, and there is a tremendous concern about postoperative morbidity which has also dramatically been reduced.

We omitted routine pelvic lymphadenectomy, patientís who have positive nodes, as you will see, end up getting radiation therapy to the whole pelvis, we do separate groin incisions, we do give postoperative radiation for groin nodal metastases, and we also give preop radiation therapy for advanced disease. Now to run through management, again for stage I, itís pretty much radical local excision, and you want to try to maintain at least a 1 cm margin and if itís truly a small lesion with less than a mm invasion, it is felt that most of those patientís do not need to have lymph nodes removed. For lesions that are greater than 1 mm in depth.

For large stage II lesions, again, depending on where itís located, we do a radical vulvectomy and bilateral inguinal femoral lymphadenectomy, if there are more than two lymph nodes positive, the patientís will get postoperative whole pelvic radiation. Many institutions will also treat the groin with electron beam therapy very superficially, and the reason to do that is to protect the femoral necks. For stage III tumors, it depends on whatís involved, you can do a radical excision which often times becomes extended and you have to take the distal vagina and even sometimes the distal urethra and if you are going to treat it surgically it needs to be combined with the bilateral inguinal femoral lymphadenectomy and again, if there is lymph node involvement.

For advanced disease, again you have to individualize, add up with surgical clearance for disease sometimes involves the anus, rectum, proximal urethra and requires an exenterative procedure with radical vulvectomy and bilateral groin nodes and that particular circumstance is very important that patientís are evaluated either with MRI, CAT scans and possibly even a PET scan for metastatic disease prior to undertaking such a large procedure. The operative mortality is about 5 to 10%.

Survival is also determined by which nodes are involved and whether or not the nodes are positive or negative. If patientís have negative groin nodes, the five year survival is 90% and thatís for stage I and stage II. If they have positive groin nodes, survival drops about 57%.

If they have positive pelvic lymph nodes, it drops to 20%. Unilateral positive groin nodes is about 70% five year survival, bilateral positive groin nodes, however, drops down to 25% five year survival, and then the increasing number of positive nodes, and also the tumor diameter affects nodal involvement, lymphatic vascular space involvement and then overall survival.

A couple of slides on melanoma and a couple on Pagetís disease and then weíre done. Vulvar melanomas are extremely rare, I have only seen a couple in my career, they are reported to be most common in the 5th and 6th decade of life, they do not always need to be pigmented, they present as a mass, Nevis or mole with pruritus or ulceration and bleeding. The masses are often located in the labia minorum or the periclitoral area and as we all know the prognosis is related to size and the depth of the lesion, and there are basically three classifications based on anatomic depth of penetration.