Associate Professor of Dermatology & of Medicine/Oncology at George Washington University, Vishal A. Patel, MD talks about a rare skin cancer.
Interview conducted by Ivanhoe Broadcast News in 2023.
So one of the things that really surprised me in looking at social media is that Jimmy Buffett revealed he had skin cancer and shortly thereafter, he’s gone, and it happened so quickly. He must have really had a bad situation. Why is that different from other cancers?
Patel: Everyone should remember that skin cancer comprises a whole list of potential diagnoses. Our skin is our largest organ. There are many cells that comprise the skin and each of those cells can potentially become cancerous. The type of skin cancer you have depends on which cancerous cells we’re actually dealing with. The most common skin cancer that patients know about in the US and will probably come into contact with, either themselves or with a family member, is squamous cell or basal cell carcinoma, which is known as the non-melanoma skin cancers as a group. And the more common, worrisome type is melanoma which most people know about. On the other hand, a very rare skin cancer which Jimmy Buffett had is carcinoma or Merkel cell skin cancer. That is a much more aggressive and rare skin cancer that involves special nerve cells within the skin itself. Each of these different types have different prognosis, and that’s why certain skin cancers seem like a little something that you get cut out and you’re fine, and something else that can be just much more detrimental to a patient’s life.
It’s interesting because since skin cancer manifests itself externally, you don’t look at it and say, that mole’s going to travel to my liver, whatever the case might be. Do basal and squamous cancer types penetrate that barrier and go internally or is it just Merkel’s and melanoma?
Patel: Any cancer has the potential for distant metastases or spreading to other organs, lymph nodes, or other places. But the likelihood of that happening differs based on the cells behavior. Generally speaking, we say that basal cell skin cancer has an exceedingly unlikely chance. That doesn’t mean that it’s not possible, there are patients with metastatic basal cell cancer, but it’s highly improbable, especially the way that that cancer behaves. It’s a local cancer that causes local destruction most of the time. Now if you ignore the cancer, it continues to grow for decades, yes, that risk can go up and it can potentially spread. Squamous cell skin cancer has a higher risk of metastasis but yet it’s very small, only a small subset, and less than 5% can actually metastasize. Those tumors are just fairly aggressive from the start. They’ll either come back multiple times, they are large to start, or they have other factors that patients are worried about.
In something like the type of cancer that he had or melanoma, what we’re seeing on the skin as it manifests, what’s going on underneath that? In other words, what does that look like directly under the skin barrier? And then when it travels and affects the body, where is it going?
Patel: Any cells that become malignant and metastasized will grow locally and depending on the aggressiveness of it, it may break off earlier in the journey of the cancer or later after it’s grown to a certain time point. That’s the difficulty of knowing how cancer is going to behave. Whether it breaks off right after it forms or it takes five years for it to break off is the challenge that we have as oncologists and dermatologists. But if you see a spot that you’re worried about, rather than delaying having it evaluated, if it’s looking different and it’s changing, those should be alarm bells that tell you that somebody probably should look at something that you don’t recognize to make sure that it isn’t something that could potentially break off and spread to another part of the body.
And the type that he had, again, it’s called Merkel, right?
Patel: It’s called Merkel cell carcinoma. As specialists in skin cancer, it’s uncommon for us to see patients with this, even as a Skin Cancer Center of Excellence. At our university, if we see one a month, that would be highly unlikely. It’s that uncommon and rare. That being said, the incidence is rising as the population ages and we also have a lot more of a sun worshiping culture among some people. We know Jimmy Buffett was certainly a sun worshiper and that puts you at higher risk. Merkel cell carcinoma metastasizes much earlier than other types of cells.
That’s more virulent as well, right?
Patel: Yes it’s much more of an aggressive type of skin cancer, meaning that out of the number of people that have it, there’s a higher chance that it will metastasize.
So is it nature versus nurture, or is it partially genetic, other environmental or how does that work?
Patel: It’s a personalized risk assessment. It’s all of the above. It’s a combination of your genetics and your environmental exposure. It can be a combination of both, and I try to explain to patients sometimes it can even just be bad luck if some cells decide to behave aggressively whereas others may not. But as a whole, we tell patients to reduce your risk by modifying the factors you can. If you love to do outdoor activities, wear sunscreen, wear sun protective clothing. Don’t do that at the peak of the sun. Do it earlier in the day or later in the day when the sun is not at its high so you can modify those risk factors that may put you at risk. And if you know you have genetic risk factors, family history, multiple people in the family that have had multiple types of skin cancers, even if you personally have a low risk skin cancer, let’s modify those risk factors so one of these more aggressive ones can’t pop up in the first place.
It’s a good question, that’s a great segue. So if you have the lower risk, basal and squamous, then is it connected to these other types, the Merkel and the melanoma?
Patel: What I tell patients is that the cells themselves are not necessarily in the skin, they’re physically connected, but each of the cell populations are individually different. That being said, they are all in the same playing field. If you’ve had sun damage that’s affected the basal cells of your skin that can lead to basal cell skin cancer, and you’ve likely had sun damage to the cells that can make up melanoma, or that can make up Merkel cell cancer. That doesn’t mean that if you’ve had a basal cell you’re going to definitely get a melanoma or definitely get a Merkel cell, but clearly you’ve had exposure of damaging sun over a long period of time that these cells are at risk. That’s why it’s important that if you are monitoring your skin after you’ve had one and if something new pops up, have somebody take a look at it.
I know we don’t know his case specifically, but in general on Merkel, what is killing the patient? What’s leading to the early death?
Patel: Merkel cell, just by its behavior, tends to metastasize at a higher rate and at an earlier time point. As opposed to basal cell or squamous cell cancers which tend to grow locally and then continue to grow and can eventually break off, in melanoma and Merkel cell, that tends to occurs at an earlier time point. In melanoma that happens in a smaller fraction of melanoma patients as a whole, and that varies depending on the aggressiveness of the tumor. But in Merkel cell, it happens in a much higher percentage. What’s unusual is that patients can be treated for Merkel cell and be doing okay, and then a few years later find out that it had spread to multiple different sites they just didn’t know. That’s the difficulty with Merkel cell carcinoma.
I see, so it’s silent as it’s spreading, right?
Patel: Correct. You may not know until it has spread and grown somewhere that a tumor is in the liver or it’s in another organ and now it’s causing other problems but it was there the whole time. With Merkel cell we take that a more aggressive approach than we do with other skin cancer. We will be aggressive about looking at other sites when you initially present because we know that there is a higher risk of metastasis with that cancer. But as a whole, there are patients that will do okay actually. What’s unusual is that even when there are patients that we think are going to do really well, perhaps three years later the cancer may come back in different area. So for these patients with aggressive cancers, it’s important to follow up long term with your oncologist or your dermatologist so that we can monitor for that. If there are any signs that it’s coming back, we can treat it early and aggressively with the other systemic whole body therapies that are available.
So externally as you’re looking at a Merkel cell patient, you’re not seeing any red flags in terms of color, internal color or whatever is manifesting on their face or wherever they have the initial cancer site can’t really tell.
Patel: We tell patients with Merkel cell cancer that if anything seems out of the ordinary, I want to know because it can be so silent. It can be hidden that it’s coming back in different areas. Anything could potentially be that the Merkel cell has spread somewhere. We want to thoroughly evaluate any potential concern. That’s not always the case for other skin cancers, just as a whole because we’re not as worried or as suspicious. However if there’s something that’s weird on the arm or on the leg, we want to make sure that that’s not a Merkel cell that’s spread off to that area. If your stomach doesn’t feel well or you’re feeling other vague symptoms, we’re going to work that up thoroughly to make sure that it hasn’t spread to other organs because we know Merkel cell can be more aggressive.
How does it look different to the eye than the other cancers?
Patel: Squamous cell or basal cancer most commonly looks like spots that just won’t heal. They’ve been there for a while. They’re slow growing, they’re rough, and they may be bleeding. The little pimples, bumps, growths that won’t go away, and they all tend to look the same. Melanoma typically come from what we colloquially call moles, but are known as nevi, or spots of melanocyte collections that have transformed. They look like dark brown spots that are irregular and growing. The challenge with Merkel cell is that it can look like anything really. They can be skin colored, they can be red, they can be big, they can be small. They can grow and look like a cyst or a boil. They can be underneath the surface of the skin. And that’s the challenge of diagnosing Merkel cell as a whole. You may not realize what it is and sometimes it can go on while it is growing and potentially spreading.
But do you know as soon as you see it, oh my God, that’s Merkel?
Patel: Certainly there are lesions that are suspicious for Merkel cell. We just say that it doesn’t fit like other skin cancers and it just looks like a red, hot and angry tumor. It may have blood vessels over it. Potentially, it can look like any variety of both benign and malignant appearing lesions. I always come back to saying if there’s something new to your skin that doesn’t look like what you have on the rest of you and if it’s something that is standing out, somebody should look at it. That’s the best approach to take. If you have multiple spots that look like each other, that’s probably reassuring. But if you have one that stands out, whatever it may look like, you should have somebody look at that.
So patient X has a Merkel cell carcinoma – what is the next steps for this patient?
Patel: It is standard protocol for our patients to have surgery (either wide local excision or Mohs micrographic surgery) for an aggressive cancer like a melanoma or Merkel cell cancer as well as have a lymph node biopsy done at the same time. They will then have a plastic surgery reconstruction done after that when we confirm that the margin is totally clear, They’re are essentially considered clear of the disease at that time point, pending the evaluation of the lymph node. In Merkel cell cancer, because of the aggressiveness of the disease, oftentimes we will add radiation therapy to the site or even potentially may do further surgery if we found cancer in the lymph node. And if the cancer returns or spreads elsewhere, we will consider systemic immunotherapy.
END OF INTERVIEW
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