Vulvar Cancer: Often Misdiagnosed as an Abscess

MD Anderson Cancer Center
Date: 09/19/2011

Lisa Garvin: Welcome to Cancer Newsline, a weekly podcast series from the University of Texas, MD Anderson Cancer Center. Cancer Newsline helps you stay current with the news on cancer research, diagnosis, treatment and prevention providing the latest information on reducing your family's cancer risk. I'm your host Lisa Garvin. Today our subject is a gynecologic cancer, vulvar cancer and we have two guests to address that today, Charles Levenback is a Professor of Gynecologic Oncology and Reproductive Medicine and Dr. Anuja Jhingran is a Professor of Radiation Oncology, both of them here at MD Anderson. Welcome to you both today.

Charles Levenback: Thank you.

Anuja Jhingran: Thank you.

Lisa Garvin: Let's talk about--let's have some basic anatomy first. What is the vulva?

Charles Levenback: The vulva is the external part of the female genitalia, so it's what you would see if you look in the mirror, if you held the mirror to the vulva.

Lisa Garvin: And vulvar cancer is rare, it's only about 4,000 cases a year?

Charles Levenback: Correct. Only about fewer than 4,000 patients a year have vulvar cancer.

Lisa Garvin: And what is the morbidity on that?

Charles Levenback: Well, vulvar cancer if it's detected early, which usually means a biopsy is highly curable usually with surgery alone. A lot of the severe consequences of this cancer is associated with patients who are diagnosed with advance disease either a large tumor on the vulva or metastases to other locations primarily the groin.

Anuja Jhingran: It's interesting because it is a case--this cancer can come in two different age groups sets, there's an early age group which 40 to 50's and there's older age group which is the 70 to 80's. So, you have to realize that the 70 and 80 year olds maybe embarrassed and not tell family members that they have a lesion that they feel so it could be pretty large by time we see it. The other thing is because it's a rare cancer, a lot of doctors think it's an abscess and may treat it with antibiotics for several weeks, months before they biopsy and find it, so a lot of times we will see it late because of those two big reasons.

Lisa Garvin: And what are the symptoms of vulvar cancer?

Charles Levenback: So common symptoms would be burning or itching, bleeding especially on contact. It cannot be overstated the importance if a woman is having vulvar symptoms and especially if they're not relieved in a short period of time or relieved by simple medications, such as an antifungal cream or medication. It's imperative to see a gynecologist and if there's a visible lesion, do a biopsy. Once again small tumors we cure with a very high and reliable rate with local surgery that preserves organ and sexual function. It's the patients who have advanced tumors that we really have problems and are more likely to lose sexual function as part of the treatment.

Lisa Garvin: Would they see or feel a lump, is that possible?

Anuja Jhingran: Yeah, in fact it feels like a boil and that's why some, like local physicians will treat it like an abscess because it feels like a boil. They can feel it a lump, they can't--definitely.

Lisa Garvin: And do they often present to you a diagnosis in early stages or is it more common to see them in later stages?

Charles Levenback: I would say it's more common to see patients actually with early tumors. As Dr. Jhingran mentioned there are kind of two groups, younger women who their invasive vulvar cancer is frequently preceded by a pre-invasive diagnosis. And these patients generally seem to be a little more--have a little better, more likely to have seen their gynecologist. They are going to a gynecologist on regular basis for other things, you know, reproductive, health issues and so, a biopsy can diagnose their disease when it might be minimally invasive, a few millimeters, and those patients we have really good success with.

Anuja Jhingran: There's also two different point etiologies for the vulvar cancers, so the younger patients tend to be always HPV positive. The older patients there's--maybe 50-50, we are doing a research project looking at that to see how many of them are really HPV positive and how many of them are HPV negative. So, for the older patients it may not be correlated with HPV, but majority of the younger patients it is correlated with HPV.

Lisa Garvin: And is, so HPV is one of the risk factors and is it a particular strain of the HPV?

Anuja Jhingran: It's the same strain as for cervical cancers, so 16 or 18 usually.

Lisa Garvin: Are there any risk factors other than HPV for vulvar cancer?

Charles Levenback: Well, HPV is human papillomavirus, so the virus is basically a sexually transmitted disease. So, the kind of risk factors that there are for cervix cancer: young age of onset of sexual activity, multiple partners, high risk partners, are all risk factors for vulvar cancer. Smoking appears to be something that enhances the risk of developing vulvar cancer. So, to say those are probably the major thing.

Anuja Jhingran: Yeah. For the older women we don't know, so it's--

Charles Levenback: There's kind of a theory about chronic inflammatory change as kind of being a prodrome to developing cancer. There are some other skin conditions, such as lichen sclerosis which people have tried to associate with the risk of vulvar cancer, even herpetic infections, but the only thing that's really very clear is infection with human papillomavirus.

Lisa Garvin: Now, should a gynecologist catch these as a matter of course during the typical annual pelvic exam?

Anuja Jhingran: Definitely, but the problem is because it's so rare they rarely see it, so they don't know what they're looking for. And especially it's interesting for younger patients like my gynecologist would ignore it because he goes, well, you're not going to get it because it's a disease of the old age, you know, and so it's an interesting concept. So, I think the older physicians that are out there have this theory in their head that you know, this is an age that you would get it and this is the age--but it is so rare that I think a lot of them just miss it.

Charles Levenback: The thing that we repeat over and over again to doctors in training when they come through our service here at MD Anderson is the importance of having a high index of suspicion and when in it doubt do a biopsy. So, a biopsy is uncomfortable for the patient. Nobody wants to have one if you don't want to--if it's, you know, if you don't really need it. On the other hand, missing a tumor and having a larger, more advanced has such serious consequences that a biopsy really has minimal risk and it's always kind of a cautious or a safe thing to do.

Anuja Jhingran: The big thing is you have to do a good gynecological exam. You have inspect, both the external and internal before you just put a speculum in them. I mean, a lot of physicians feel that the only pelvic exam is to put the specimen in and examine the cervix. Well, that's how miss vaginal lesions and that's how you miss vulvar lesions. So, it's really important when you're doing a good gynecological exam that you inspect the entire area and that's how you would not miss a lesion like this.

Lisa Garvin: And we talked about surgery--well, actually let's back it up just a little bit, the incidence in metastases is high, low, medium?

Charles Levenback: So the incidence of metastasis is directly related to the depth of invasion of the tumor. So, if a tumor for example is less than a millimeter the risk of metastasis is so low that we don't even do a surgical evaluation of the groins. However, as it gets deeper, the risk goes up and for example for patients with over 5 millimeter of metastasis, the risk of having spread to lymph nodes is probably in the 30 to 50% range, so the depth is a really important factor.

Anuja Jhingran: It's also very methodical, so it goes first to your groin nodes and then it goes to your pelvic nodes and then it goes higher. So, vulvar cancer is very, very picky. It will first go to the groins and then pelvic and then higher.

Lisa Garvin: So, it doesn't have distant metastases to like the lung or the brain?

Charles Levenback: Extremely rare, it can happen, but it's just extremely rare. At that outset that we would see a patient with vulvar cancer at the time of their presentation has spread to the distant sites that you mentioned.

Anuja Jhingran: Without having nodes that are already positive.

Charles Levenback: Right.

Lisa Garvin: And do you do a sentinel lymph node biopsies?

Charles Levenback: So, sentinel lymph node biopsy has been a real interest of ours here at MD Anderson for several years. This technique was pioneered in patients with cutaneous melanoma and involves injecting radiocolloid and a vital blue dye around the tumor and then identifying the sentinel node or the first site of metastasis. So, we have developed the technique to apply to vulvar cancer patients of injecting around the tumor and then finding the sentinel node in the groin. This as with other disease sites, this is a big blessing for our patients because in the previous paradigm we were doing lymphadenectomy, inguinal, femoral lymphadenectomy to identify the node positive patients and that was associated with a very high risk of lymphedema or chronic swelling of the lower extremity. So, now we have this technique that we can instead of removing the eight to ten lymph nodes from your groin, remove maybe one or two lymph nodes from your groin and get actually better information about whether they spread or not without causing lymphedema. So, it's really been gratifying to be able to extend this to patients with this tumor.

Lisa Garvin: And we talked about surgery in some cases being the primary treatment, but Dr. Jhingran, you use radiation on vulvar cancer patients as well.

Anuja Jhingran: Correct and it depends on the size and the depth of the lesions and it also depends on where the lesion is. So, if the lesion is close to an organ that you want to spare like the clitoris or the urethra or the anus, and if you try to do a resection and the margin turns out to be very close, you rather do a radiation preoperatively to try to shrink that tumor so that you can spare organs like those three that I mentioned.

Lisa Garvin: So, it is done in advanced to surgery to shrink.

Anuja Jhingran: It can be done in advance of surgery to shrink or if it is a very large tumor where surgery may not even be an option, we can try to do--treat them with curative intent with radiation therapy.

Lisa Garvin: What is the most common, are you using gamma knife, are you using IMRT?

Anuja Jhingran: We are using IMRT now in most of our vulvar cancer patients because we can spare normal tissues especially the soft tissues that have to get extra doses especially in the legs as well as in the other areas. So, we are using IMRT in most of our vulvar cases, that also we will use it for postoperative patients if they have positive nodes, they are guaranteed to have postoperative radiation therapy.

Lisa Garvin: What is the incidence of sexual dysfunction in vulvar cancer patients?

Charles Levenback: Well, that's a great question and you know, it is something that is not easily to study or well studied and as we talked about before there is a very wide age range of patients including premenopausal patients who clearly are sexually active and elderly patients who are maybe no longer sexually active. However, our observation is that you know surgery--this type of surgery can have such an adverse effect on body image, vaginal function that most patients, that rehabilitating sexual function after this type of surgery can be difficult for a patient and their partner. And we really try and do a better job now at kind of talking about that before surgery. We do have sexual counselors available to talk to patients about this and we try new surgical techniques that kind of preserve the introitus, the opening to the vagina so that intercourse is still possible.

Anuja Jhingran: And the problem with giving radiation therapy is that it does make it very difficult because we have to treat the whole vulva and we have to treat the vagina. So, we do give them dilators, we do talk about it, but again because of the different age groups, the 80 year olds are going to tell you, I'm never having sex again where the younger patients it is important. But we do try to counsel everybody. But after radiation therapy for vulvar cancer, that is a big issue, huge issue because we are treating the entire vagina and the introitus.

Lisa Garvin: Also, there is a possibility that the vaginal opening closes up.

Anuja Jhingran: Yeah.

Lisa Garvin: Oh, so that's what the dilators are for?

Anuja Jhingran: Right.

Lisa Garvin: Oh, okay. Is that something that they have to wear for--

Anuja Jhingran: Well, then they use it, you put it in two or three times a week at night. You'll leave it in for two to three--for 15 to 20 minutes and then you take it out or regular sexual intercourse, one or the other, but it really is an issue and we are definitely not going to say that it is not because you are treating that part of the body.

Charles Levenback: Right and it is not just the actual anatomy. It is--

Anuja Jhingran: Scarring purposes.

Charles Levenback: You know, scarring, estrogen dryness, a lot of issues.

Anuja Jhingran: The advantage is this is not correlated with estrogen, so you can give them estrogen cream which we use a lot to help heal and help bring the moisture back into the vagina, but still. I mean, for the young patients who treating their ovaries, so they are--if they aren't menopausal already, you are going to make them menopausal. So, each group has different issues. The older patients because they are older, you are treating a lot of bone, you increase their risk of osteoporosis, and fractures, so I mean, these are--each group has a different side effect.

Lisa Garvin: What sorts of treatments are we looking at or what's down the road? Obviously, you have such a tiny patient population to work with. Have you been able to, like, are there things like proton therapy or robotics or targeted therapies that you are looking into?

Charles Levenback: Well, the surgery for this does not enter a body cavity, so, the robot really doesn't help us here. I think it is developing and expanding sentinel node biopsy that is a big part of what we do. I feel like we work more now with the other surgical sub-specialties especially the plastic surgeons at trying to preserve, you know, sexual functions in patients who are having this type of operation because the tumor is rare, there aren't large randomized studies. And in fact, Dr. Jhingran and I both participated in a Gynecologic Oncology Group which is a national consortium and that the GOG has been successful at completing a sentinel node protocol with over 500 patients, but treatment protocols have been very difficult in the group to complete. So, a lot of the innovation comes from places like MD Anderson where we more patients than the typical place and can kind of try and develop new strategies.

Lisa Garvin: And about proton Dr. Jhingran, a lot of prostate cancer patients is being treated with it.

Anuja Jhingran: Right. So, actually we are looking in doing a protocol using protons. We are going to do it with Mass General who also has a proton center, but the problem with vulvar cancer as well as any cancer that's in the pelvis besides prostate is that there is gas, because if you are treating the pelvis there's a lot of gas. And gas changes the depth of protons, so as your bowel gas moves, it changes the depth of the proton. So it's difficult unless you're only treating the vulvar and the inguinal nodes you may be able to get away with it, but the majority of the patients don't just have the vulvar and inguinal nodes that need to be treated, but the pelvis nodes as well and that's where protons are hard. We could use protons as a boost to the vulva which I think would be very helpful, and like I said we are looking a protocol with Mass General to see if we can do this, but we are evaluating plans to see if we can get away with it. But I think the big innovation for vulvar cancer right now is sentinel nodes which is being investigated is now where the GOG is looking in doing a international trial looking at sentinel nodes. So, that's one of the biggest innovations we have because we have women with big lives just from no dissections, so sentinel nodes is big in vulvar cancer. We've just completed a Phase II trial in GOG looking at concurrent cisplatin and radiation therapy that had good results. It was just presented last year.

Charles Levenback: Interestingly, one subgroup of vulvar cancer patients that we've been--feels like we've been seeing more of our patients with melanoma of the vulva, and so we don't usually think of--we usually think of melanoma as occurring in sun exposed areas, but there are patients who have vaginal vulvar, anal melanomas and along with our colleagues in medical oncology there are several innovative trials looking--one of which is looking at a drug named dasatinib in the treatment of these patients and the other is they are harvesting tumor to try and develop the so-called tumor immune lymphocytes. So, this is one area where there's a lot of interests and the kind of melanoma from the other sites overlaps with gynecologic melanomas and it's going to be very interesting to see if some of the innovation from melanoma can be applied to our patients.

Anuja Jhingran: I think the big thing to remember though for vulvar cancer even when they're 6 or 7 cm in size and we do see a vulvar cancer are 6 or 7 or 8 cms in size, they respond very well to radiation therapy. And we can cure even a 6 or 7 cm vulvar cancer that hasn't metastasized to the pelvic or the common iliac nodes, so if it's a locally advance vulvar cancer, we can cure these patients. So, you know even those patients who really do go all the way and we can definitely cure a 7 cm or 8 cm lesion.

Charles Levenback: And patients with smaller tumors, the patients with the 1 or 2 centimeter tumors we can operate on them and for the vast majority do so in such a way that's not disfiguring and preserve body image and sexual function.

Lisa Garvin: Good information and encouraging information for women who may have this cancer. If you have questions about anything you've heard today on Cancer Newsline, contact askMDAnderson at 1877-MDA-6789 or online at www.mdanderson.org/ask. Thank you for listening to this episode of Cancer Newsline, tune in next week for the next podcast in our series.