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Vulvar Melanoma
Vulvar Melanoma
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Video Transcription
I just hope it won't get really weird talking in one screen and sharing in another because I'm going with two different internets to get everything true. Okay, great. So let's go. We are going to talk about vulvar melanoma today. When you check the articles and read about it, they will say like VM or VVM, which is vulvar and vaginal melanoma. That's what you will find. So we know that vulvar cancer is one of the less common gynecologic cancers and the age of the diagnosis is about 68 years old for the vulvar cancer, but you will see that it doesn't apply to melanoma. Most patients with vulvar cancer are diagnosed in early stage and that's not different for melanoma and the survival rate is about 72%. The vulvar cancer, we have many histologists, but the most common one is squamous cells and the second one is melanoma and that's why we're going to talk about it today. So melanoma is the second most vulvar cancer histology and it accounts approximately 2 to 10% of primary vulvar neoplasms. Median age is approximately the same of vulvar cancer, but it's really different from cutaneous melanomas that presents in other sites, which develop usually at the age of 45. So we have to be careful with that. We will see that the vulvar melanoma is actually a melanoma and not really a vulvar cancer, but we are going to see as well that the vulvar melanoma has special characteristics different from the cutaneous ones. It's usually a pigmented lesion, but there are melanocytic lesions in the vulva as well. The special part of this kind of melanoma, the vulvar and vaginal one, is that they are unrelated to chronic sun exposure and damage of ultraviolet light, which is the physiopathology of the other kinds of melanomas or many of them the cutaneous ones. The signs and symptoms of the vulvar melanoma are really similar and they start pretty much the same as the vulvar cancer, which is asymptomatic or oligosymptomatic. You only have the lesion over there and it's a small one. Sometimes the patient doesn't even see. It goes to a routine screen of PET tests or going to the gynecology, the once a year visitation and the gynecology is spotted, but it can also have pruritus, which is one of the most common ones, bleeding, ulceration, pain, and tenderness, which is pretty much the same as the vulvar cancer. When we talk about vulvar cancer, we have the labia majora as the most common site. Here we have the clitoris and the labia minora, so we have to be cautious with pigmented lesions that are in those areas. And when we talk about the vulvar melanoma, that's why I say it's not really a vulvar cancer is because the vulvar melanoma is diagnosed as a cutaneous melanoma and it's kind of treated as a cutaneous melanoma. So it's not really a vulvar cancer, but it is in a place that we gynecologists, oncologists know a lot better than some other specialties. So the diagnosis, and we have to remember that up to 10% of women will have pigmented lesions, vulvar lesions, along their lives and the majority are benign. So we do identify them and diagnose them with the ABCDEs similar to other dermatological lesions all over the body, and just the same as the cutaneous melanoma, which is with the asymmetry of the lesion, the irregularity and the borders, the color, of course, the diameter, usually greater than six millimeters, and if it is evolving. So if you do get a patient with a lesion in the vulvar area, especially in the clitoris and the labia minora, and you still don't think it's the time for a biopsy or you don't still think that it would be a malignant lesion, you should ask her to come before her usual time, like one year, you ask her to come before and take a look if there are early signs of evolving, of changing in size, shape or color. And there is no diagnosis at all without the biopsy, without the histopathologic, without the anatomopathology, without our friends in pathology, we don't have a diagnosis. We have only a hypothesis, which are made with the ABCDEs. And it's really interesting that the vulvar melanoma has unique molecular features, really different from the cutaneous melanomas. The immunohistochemistry, it's usually similar, and we have those markers that are pretty much the same that Gustavo and Dr. Ebica asked when we had the case, the suspicious case of the vulvar vaginal melanoma, and the molecular features are these ones that I put here. I actually don't know a lot about them, and so if Gustavo is still there, he can complement me. But we have to look at them, and they are special, because they do have target treatments for them. So it's the BRAF proto-oncogen, the NRAS mutations, and the PD-L1 and PD-1 that are really frequent. And besides that, we have a high rate of CKIT mutations. I know maybe in Uganda setting, you don't have all these kinds of immunohistochemistry. I know in Scola Paulista de Medicina, where I work, we don't have all of them. So it might be really hard to get all these molecular features known in the cancer that you are treating. Again, the staging, you stage like a melanoma, like a cutaneous melanoma, not like a vulvar cancer. We are not going to stage based on FIGO. We are going to stage based on TNM and AJCC, and we are going to use Breslow's and Clark's grades and thickness to have a better prediction and to know which treatment we are going to do. So if we stage like the AJCC, we have a far better predictor of survival, and we have the GOG73 study to show us that the AJCC is better than the FIGO staging, because melanoma is not really a vulvar cancer. And this is just for you guys to check, to take a look, because if we are not working with this every day, I think it's pretty, pretty good to get it all in our heads, because it's not a really easy staging system with a lot of variations in just really minimal millimeters, right? So we stage as the AJCC, and the 8th Addiction came with some other features, and that's the one we should follow. The treatment, we will treat like a melanoma, like a cutaneous melanoma. We are not going to treat like a vulvar cancer, but it is pretty similar. It's not far off our reality. But in the special, the treatment, the primary treatment, and what we have to focus is the surgical excision. It is surgery. It is the best treatment. It is the primary treatment, and it is, which probably is going to give us the best prognostic factor for the patient, besides the lymph nodes that we are going to see in a bit. So when I say surgical excision, and I say that we are going to treat as a cutaneous melanoma, it's because we are going to use the tumor thickness and the brassel thickness to determine how much margin do we have to do for this surgical excision. So we have the GOG72 trial that I have already talked about before, about FIGO and the AJCC staging. And this trial also indicated that the outcomes of the local excision is better than the vulvectomy or radical vulvar surgery. And that's why I like to say that the vulvar melanoma should be treated like a cutaneous melanoma and not like a vulvar cancer. That is kind of changing the vulvar cancer as well. Thank God we are having smaller surgeries with greater or same results and having a lot less comorbidities and complications after the surgeries. Which is very interesting here is that even before we had the sentinel lymph node biopsy before the vulvar cancer, which is well established nowadays, we already did for the melanoma, the vulvar melanoma. So melanoma is one of the first cancers, the cutaneous melanomas, to do the sentinel lymph node biopsy with great prognostic factors, great sensitivity and they also have always been here in the vulvar melanoma and we are following the sentinel lymph node in many more places like breast cancer, which is well established, and we are going through gynecology oncology as well in vulvar was the first site, now cervical cancer and endometrial cancer as well. In women with clinically apparent lymph node metastasis, that means when you see it, it looks like it's already positive, you should do a dissection and excision therapeutically. It's not really needed a systematic full lymphadenectomy, but you should excise, especially after excluding this metastasis. I would like to show you a case which is quite similar of an amylo-nocytic, but there is an excision biopsy before, so it had a little color before and we did follow the Breslow and Clark's grades and thickness to do this excision. So we did a hemivolvectomy here and you can see we have a few margins that are a little bit off limits, which are the superior and the inferior, and I will tell you guys that what we recognize here in Scleropolysis Medicina is that maybe sometimes it is better if you do a greater margin, but you do a better closure and do not to do any patches and any plastic surgery specifically in this place, because the vulva is a place that has a high incidence of baseness of the stitches and the operatory sites. So we went through and got a pretty similar as a vulvectomy, but as I said, two centimeters margins because of the thickness and the size of the tumor and we enter into the fascia as we can see and the closure is really good. Every time we deal with the vulva, we like to drain the site in order not to make a big seroma or get a higher, you get a better result from infections. And I will show you guys how it became and the two months post-op and it was pretty good. We did the sentinel lymph node as well. We could use the two techniques, the blue patent and the technician. They marked for us the place and we went with the both characteristics and found the blue lymph node, which was the one that had the more captation of the technician as well. And that's it. And it was a beautiful surgery, the patient is great. I will just show you another surgery, which we did not do this kind of extensive margins. It's a posterior, this is a squamous cell, but just for the fellows to get a look. When we do not do this extensive margins, we might need to do some patches. So here we did a dip, we call that a dipoplasty and we had to rotate the skin in order to be sure to make a great recovery. But I do think it gets a lot more tense and the results are not that good. Just wanted to show you guys that this was a squamous cell. When we talk about the adjuvant treatment, always remember surgical treatment, it is our primary treatment. We have all treatments are for melanoma as the cutaneous one, but we have little information. We don't have many articles and many information that's specific about the vulvar melanoma because they do not talk about, they do divide cutaneous from other kinds, but they don't usually say if it is a VVM or if you're dealing with an acro or any kind of other mucosal melanomas. So we have always targeted treatments and the two, the ones that are most used in cutaneous melanoma with better responses are nivolumab and pembrolizumab, especially if you have nodal involvement. We have also checkpoint inhibitors and tyrosine kinase inhibitors. We don't have here in SUS, maybe in our private practice, we can get those kinds of treatments and those kinds of drugs, but when we talk about the primary care system, which is free here in Brazil, which is called SUS, we would have to go to cytotoxic agents like carboplatin and flacoprotexil or temozolamide, which are the ones that should help or treat melanoma. But as I already said, the prognosis is not that good. So when you go to vulva and vulvovaginal melanoma, you can see that it's always surgery. If you expect, well, you always choose surgery and if not, you go to systemic therapy. Radiotherapy has a part of the adjuvant treatment, but it's not that great as in squamous cells. The prognosis overall is a poor prognosis. In five-year rate survival, it's about 46% compared to cutaneous melanoma. And what we have, predictors of outcome is age, ethnicity, stage of diagnosis, tuber thickness, lymph node status, histologic subtypes, mitotic counts, ulceration, which it counts as the Brazil, and lymphovascular invasion, as well as perineural invasion. And in the latest multivariable logistic regression analysis, lymph node status and mitotic count appear to be the most important predictors for survival. If you do get full surgical excision and you get this patient all treated, you should follow her up every four to six months and consider CTs or PET scans in the same amount of months, four to six, or in the most, every 12 months or as annual. I hope I could get all the information through in a small amount of time. And that's my email if you guys need to check in or have any other questions. Thank you very much.
Video Summary
The video discussed vulvar melanoma, which is the second most common histology of vulvar cancer. Unlike other types of melanoma, vulvar melanoma is not related to chronic sun exposure. Symptoms of vulvar melanoma are similar to vulvar cancer, including pigmented lesions, pruritus, bleeding, ulceration, and pain. The diagnosis is confirmed through a biopsy. Molecular features of vulvar melanoma include BRAF proto-oncogene, NRAS mutations, PD-L1, PD-1, and CKIT mutations. Staging is done using TNM and AJCC stages, and treatment is similar to cutaneous melanoma, with surgical excision being the primary treatment. The GOG72 trial found that local excision had better outcomes than vulvectomy or radical vulvar surgery. Sentinel lymph node biopsy is also performed. Adjuvant treatments for vulvar melanoma include targeted therapies such as nivolumab and pembrolizumab, and cytotoxic agents like carboplatin and flacoprotexil. The prognosis for vulvar melanoma is generally poor, with a five-year survival rate of around 46%. Follow-up includes regular check-ups and imaging scans.
Asset Subtitle
Maria Gabriela Baumgarten Kuster Uyeda
October 2023
Keywords
vulvar melanoma
symptoms
diagnosis
treatment
prognosis
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