Dr. Caroline Piggott addresses moles in children including a discussion of benign lesions as well as worrisome lesions that may need further diagnostic work up.
well, welcome back from the break and to take us up to our lunch break will be the course co director Hugh Green weight. Uh Dr Greenway really needs no introduction actually. He has trained over 30 fellows in Moe's surgery and cutaneous surgery. He actually trained with fred mO's in MO's surgery and has done over 31,000 surgical procedures at the Scripps Clinic In 1989. He and I were having lunch in the scripts cafeteria and he suddenly pulled a napkin out of the dispenser and said, you know what we need to do. We need to have a course on cutaneous malignancy and started outlining the course and here we are, 31 years later. So Dr Greenway take it away. Thanks terry. Thanks for directing of course all these years. One of the most popular talks and concerns comes up with what about nev I and melanoma in Children or kids? So today dr Carolyn Piggott who is one of our adult and pediatric dermatologist that scripts clinic is going to cover that She treats all types of skin conditions and Children and as a particular interest in melanoma and acne. She's active in screening for both kids and adults. She was raised in Vancouver Canada and then attended Harvard and she's been here in San Diego for about 12 years. Um in her spare time. She enjoys figure skating and concerts and she's really a fan of the san Diego Padres baseball team and Caroline. The team should do better. They've spent more money than any other baseball team looking towards next year. So we really appreciate you taking time to give this most popular lecture on all we need to know about moles and melanoma and kids. Dr piggott. Can you hear me okay? Great. Thank you for the introduction dr Greenway and thanks for having me back again this year. Um, I let me just make sure my cursor works okay. There we go. So here's my little padre. I'm glad you mentioned that. This is my son Michael, future padre. Um, so what we're going to talk about today are moles and melanoma and Children. And the good news is the vast majority of cases that I see are completely benign and I spend my time reassuring parents. Um, so the good news is that childhood melanoma is very rare. Um, There are special cases with risk in Children of course. Um, spits nearby in particular um, as well as large and giant sized in general nearby. Um my job a lot of it is actually on educating parents on how to prevent melanoma. So what we're going to talk about today is what to worry about in the clinic um, is something benign versus malignant. Um then we're going to talk about melanoma and Children uh, followed by spits nearby and then I'll finish off with congenital nearby. So what to worry about in the clinic. Um, so this photo right off the bat is a very scary looking leisure and this is a photo of a very uh terrible melanoma in a child. Um you can see that there's a pink popular with significant ulceration in the center and this is indeed a case of pediatric melanoma. Um So how what do I look for? So I go by of course the standard A. B. C. D. Rules that we use in adults. Here's a photo of the little A. D. Pamphlet that we have in our clinic. Um And this is especially important in older Children and teenagers. Um We look for a symmetry, funny borders, dark colours or multiple colors large diameter and evolution. However, um there was a great publication out of U. C. S. F. Um and it was published in 2013 entitled pediatric melanoma results of a large cohort study and proposal for modified A. B. C. D. Detection criteria for Children. And this is this was done by kelly Cordaro. Um And what they did is they had they looked at a cohort of 60 Children at UCSF under the age of 20 years who were diagnosed with melanoma. And then um what they looked what they looked at when they analyzed this cohort is that um the Children who had melanoma who were 10 years and under 60% of those melanomas did not present with a conventional a. b. c. d. rules. Um in this cohort uh those who are aged 11 to 19 years, 40% of the patients with melanoma did not present with the conventional A. B. C. D. E. Criteria. Um In this cohort also 10 out of the 60 melanoma patients passed away And nine out of 10 of them were over the age of 10 years. And of these patients who passed away, 70% of them had a melon arctic lesions, a lesions without pigmentation. So based on these findings um uh kelly Cordaro proposed a new pediatric A. B. C. D. Rule. And in this the A. Stands for a melon arctic, the B stands for bleeding or bump. The C. Stands for color uniformity and the D. Stands for de novo or any diameter. And it's not that we're supposed to throw the old A. B. C. D. S out the window but use these in conjunction with the conventional A. B. C. D. S, particularly in Children who are younger under the age of 10. Another tip I would like to give is to trust the parents intuition when someone brings you their child, they're they're bringing you the best they have to offer. So if a parent is concerned, even even if your gut tells you that something is benign, respect what they have to say and consider biopsy based on a parent's concern. So moving on, let's talk about melanoma in Children. So the good news is that the incidence of melanoma and Children is very rare, especially before puberty. However there out of all the melanomas, 1-2% of them are in Children uh in terms of risk factors for melanoma and Children. All the risk factors are very similar to those we look at an adult. So, some of the things that I look for a family history of melanoma, um is that a larger, giant sized congenital nevis? Are there many nearby including a typical appearing nearby? Um does the child have a fair skin type? Um I ask about a history of blistering sunburns and then of course there, are there any underlying genetic anomalies such as XP. So one of the best um studies that we have in the United States are based on something called the Seer database. So, the essence, our surveillance epidemiology and end results. And this looks at this this co heart looks at risk factors for pediatric melanoma. And in the United States, risk factors include caucasian race, female living in Seer registries with low UV B exposure, I like Northern States closer to the Canadian border. Um the highest incidence rates by sex were for boys, the face and trunk and girls lower limbs and hip. And um there's actually a public, there was an initial publication by Wang. Um and that looked at us data through 2005 in the United States. And what was worrisome in this is that the melanoma incidence rate was increasing in all ages, including pediatric adolescent and young adults. However, Um in 2020 there was a new publication on the seer database and this includes a timeframe from 2001 to 2015. Um and this of course includes all ages of patients diagnosed with melanoma. In the United States. In total, there are almost a,188,103 cases of invasive melanoma diagnosed. Um and I looked at in particular the most recent year, which was 2,015 in the seer database. Um in 2015, 80, 3000 cases of melanoma were reported in, this includes all ages. Um, if you look at the actual ages, specifically, 67 patients in 2015 diagnosed with melanoma were under the age of 10. So, fortunately that number is very low, 251 patients were diagnosed with melanoma between the ages of 10 and 19 And 1,973 cases were diagnosed between the ages of 20 and 29. Um between 2006 and 2015, the overall incidence rate of melanoma increased um From 2 to about 200 to 229 cases per million person years. What the only encouraging news though, which I thought was great is that there was actually a statistically significant decrease in melanoma incidents in the United States for age 10-19. And that was down 4% for men and 5% for women. And this, the this number was also decreasing for patients from the age of 20 to 29 Between 2006 and 2015. Also it was found that young adult women had twice the risk of melanoma as young adult men. Um But I thought it was very encouraging that overall the there was a decrease in melanoma incidence for young people. And the hypothesis is that perhaps public health efforts may be influencing melanoma incidents, but we were just really don't know. Um There was also a great study in 2000 and 18 on pediatric melanoma and melanoma prone families. Um And this was a study done through the NIH and they looked they had a total of 60 melanoma pro pro families that they studied and to to fit into this study you have to have at least three or more melanoma patients in your family. Of those 60 families, it was about 50 50 split on those who were C. D. K. Into a positive and negative and in the study they look both at the age and the number of melanoma is diagnosed in each patient within these families. What was very interesting is that regardless of C. D. K. Into a status in melanoma chrome families, there's about a 6 to 28 fold higher proportion of pediatric melanoma compared to the general population rates. Um Also of these 60 study families um the families who had C. D. K. Into a positive genetic status. They had about 11% of the pediatric melanomas compared to 2.5% for the C. D. K. And C. D. K. Into a negative families. So when I took from this is how important it is for patients who are in families where there's multiple melanomas um Perhaps it's imperative for us to start screening younger. Um I tell most families the best time to start screening is in the teenage years but in a family with multiple melanomas I actually have started to do annual skin cancer screenings even on little ones so treatment of pediatric melanoma. So surgery is the mainstay of it um Just like in adults. Um we do wide local excision of the primary. Um There's also cases where we do sentinel lymph node biopsy. And um because pediatric melanoma is so rare we basically just follow the adult parameters. So we look at the melanoma death. Is there ulceration etcetera? Um Having a positive sentinel lymph node does have prognostic value of in Children. Um uh lymph node dissection is controversial if you do have a positive sentinel lymph node biopsy. Um and in fact complete no dissection is no longer recommended in the absence of clinically or radiographic lee positive notes. Um Instead it's common that nodes are followed by ultrasound instead. Um for systemic therapy also because of the number is so low we have very limited studies on on what systemic therapy is best. Unfortunately Children are excluded from most melanoma trials. Um in the in the past the most common treatment was interfere on. But in recent years just like an adult immunotherapy um has been used. But all our recommendations are based on pediatric retrospective data and data from adult clinical trials. So there's no sort of standard of care usually for patients who have metastatic melanoma. They're managed by a tertiary pediatric academic center um such as in SAn Diego. We do it at readies. So spits nearby. Let's move on to those. Um So the following patient walks into your clinic on friday afternoon. Of course. Here they are. This is the forearm of an 11 year old girl. We see a very dark, popular I think I have a closer image. The borders are a little bit scallops Scout and the mother reported this had only been around for a few months. So sure enough, I biopsied it and it came back. I actually did a punch biopsy and the diagnosis was in a typical spits nevis. So here is another case that I have thought I believe about two years ago this is a baby under the age of one who presented with a new onset pink pop. You'll know prior treatment, no bleeding like you would expect from a pathogenic granuloma. So I did a shave biopsy with mom swaddling the baby and it came back up to nine spits nevis. So benign spits knee by the classical clinical clues that were all taught is to look at the morphology is there a symmetric arrangement of colors? We look at the structures and DeMoss copy is also very helpful. Um and it depends on whether it's pigmented or not. So for a pigmented spits nevis that's benign. We commonly see the star burst pattern which I'll show you a picture of that in a minute and there is thought to be a central area where you have homogeneous blue black pigment and there's also peripheral radio and symmetrical streaks or pseudo pots. For a spits that doesn't have pigment. You look for symmetric dotted vessels in a benign spits nevis. Um In terms of clinical, here's some photos of a bad spits nevis or in a typical spits nevis. There's two of them. The one on the left is a pink package with a central ulceration and there's significant scale as well. And the one on the right shows a pink popular with multiple, multiple colors of brown within the center and even a little bit of gray. And those both came back a typical on biopsy, not melanoma, but a typical. The only issue with DeMoss copy is there's actually a scary study published in 2015 by Lollis at all and they showed us to use these classical DeMoss copy criteria that we're all taught with caution. And they actually had one of the biggest studies of Spitz Navy that's been published. They looked at excision specimens of 384 schizoid looking lesions pre clinically In patients 12 years and older it was a multi center study between Italy Japan and Brazil. Um the good news is that um 86.7% of the lesions that looked like a spitz nearby came back as benign. The scary thing though, was they in this study to be included. You have to also have Durmus copy photos taken beforehand. And the photo I'm showing you is of that sort of classic star burst pattern I told you about. But the scary thing is these look almost identical and one of them is benign and one of them is in a typical spits nevis on pathology and you can barely tell the difference again I mentioned so for spitz nearby that don't have any pigment here's that DeMoss copy sort of classical symmetrical dotted vessel pattern. And these photographs are almost identical. And one of them is any typical spits nevis and one of them is benign. So basically what they taught us to is to use DeMoss copy features with caution. So when you biopsies that seems you'll get back a path report and somewhere um in the following histological spectrum you'll get a result. And I thought this was quite a good chart to use showing the spectrum of histological features that you can get the diagnostic term applied and the implied likely behavior of the lesion. So on the left we have the term spits nevis which is benign and moving towards the right, we have an atypical spits nevis diagnosis, which we still think to be of low risk of malignant potential. Probably benign. But moving farther to the right, we have the term atypical spits tumor or stump. We abbreviated it also known as spitz tumor of uncertain malignant potential, which has uncertain malignant potential. Um and and a higher risk of worrisome biologic potential, basically. And then finally there on the right there if you see spitzer melanoma and common melanoma, which is obviously implying malignant behavior. So what do pathologists look at for diagnosing a typical spit side to us? I'm no pathologist, but I know that things they look for are things like whether or not there's um symmetry. They look at the depth, is there a lack of maturation? They look for solid growth? They look for nuclear Play. A morph is um they look at whether or not there's a high nuclear cytoplasmic ratio. And then also very important is the presence of mitosis. Are they typical? Are they deep or are there more than six square? So in the clinic, what do you do if a spitz needs to suspected? So you look at the patient and you think it's a spitz? What do you do? So even the answer to this is historically controversial, but the general consensus among pediatric dermatologists as that it's better to do a biopsy than to observe. Um but and then the next question is so you decided to do a biopsy. What do you do? Do you shave it? Do you punch it? You excise it certainly in an older child or a teenager of excision as possible. You know, theoretically that's the best. But then the harder decision is that most kids you can't do an excision right off the bat. Do you shave it or do you punch it? So the traditional belief held is that punch removal if possible is the best and that. But of course shave biopsy is better than nothing. But we really do this on a case by case basis. And there's actually some new data out of Harvard, it's plastics, oral surgery data that was published in 2020. And it's limited because it's a rescue retrospective study. But basically they looked at 123 re excisions of biopsy proven spits nearby that had incomplete removal on the initial biopsy. So positive margins and it could be either the initial biopsy with an excision, a shaver a punch. And then what they did was they looked at the re excision specimen and to see whether there was any residual actually found. And the% 123 cases. The presence of residual lesion in re excision specimens was significantly higher when the initial biopsy was done by punch versus shape, which I thought was interesting. And so this suggests maybe even to consider a shave would be better than punch biopsy. Again this is very limited data retrospective etcetera etcetera. But it also I thought implied perhaps that clinical observation may be a reasonable approach after the initial biopsy. Even if margins are involved, if there is no evidence of clinical residual. Certainly if there were clinical residual re excision would be recommended regardless, but if there is none, perhaps it may be okay to observe the specimen or observe the lesion in real life. Um So treatment, so you have a child who has a biopsy proven spits nevis. Now what do you do again? Unfortunately there is still no consensus among pediatric dermatologists but this is generally what I do. So if the initial biopsy shows a benign spits nevis, I re excise it If possible if there is a residual lesion clinically or histological e if I can't easily re excise it. I then observed very carefully. If it's benign of course if there is no clinical residual um if I'm going to observe it, I also very aggressively educate the family on the rare potential for malignancy and I do close clinical follow up perhaps every six months or something like that. So what do you do if the initial biopsy is a spitz with a tibia? I definitely re excise it in most cases if for whatever reason I am unable to re excise it which happens very rarely. I do very close observation as well. And then certainly in a spitzer melanoma. Re excision is the utmost importance but it's really actually quite controversial weather to do a sentinel lymph node biopsy or not. Um so usually to help make that decision, I refer the patient to a pediatric oncology center where it's quite difficult. Of course in practice is are the lesions that are more borderline atypical spits tumor versus melanoma. Um, certainly I exercise them, but there's actually no outcomes data that exists with either a typical spits tumor or melanomas um documenting any survival benefit with sentinel lymph node testing. So generally in these cases we look at them on a case by case basis of course, um based on data nodal mets actually from a typical spits tumors are not uncommon, but death from widespread disease is rare. If we do decide to do a sentinel lymph node biopsy and it's positive, then the question is, do we do a complete lymph of the neck to me or not? And the problem with complete lymphatic next to me is that there is increased risk of morbidity from the procedure itself and no evidence in any data of increased survival. Um, also if lymph node disease is found um, I would definitely, as I mentioned before, consider referral to pediatric oncologist. For consideration of systemic therapy for example, interfere on versus some of the new immunotherapy agents and of course this would be off label in pediatric patients. Um and the good news is that death is very rare from spits us. But there are reports of death from a typical spits tumors in their literature, which is um, the scary part of all this. Unfortunately though very very rare. So moving on now, we're going to talk about congenital knee by um, so the following six weeks, six week old baby girl presents to your office and here's the photo. So this is the scalp. This is about a two month old baby, you can see paint brown colors, their satellites, etcetera. So this is a classic difficult case for general nervous that I think I saw this girl two years ago, fortunately we took a little biopsy of one of the darker spots. It was completely benign and we've actually observed it and she we didn't re excise the whole thing and she's doing very well. And in fact the lesion is actually lighter than it is in this photo nowadays. Um, so the most important thing to do when you see a congenital nevis is to classify the lesion based on size and how we, how we classify it is not based on how big it is when they're little, we try to project what diameter it's going to be in adulthood And in an adult we consider it a small size can generally, even if it's under 1.5 cm, it's medium size. If it's 1.5 to 20 cm, it's a large one if it's 20-40 and it's a giant size can generally, even if it's 40 cm are higher at an adulthood. Other features that are important to note is whether or not there's any satellites. Um It's also very important to palpate the lesion to make sure there's no nodule because those sometimes can be more worrisome as well. Um In terms of so how do I project, how big it's going to be an adult? So um as long as the baby, the child is no more than say about two years um ideally under one. If it's on the head, you take the measurement of how big it is in centimeters and multiply it by 1.7. Um On the lower extremities, you multiply it by 3.3. And if the general nevis is on the trunk, upper extremities, your feet, you multiply it by 2.8. And this was based on data collected on a large sample of congenital, nearby. They measured them in childhood and then they measured that again in adulthood and they came up with these numbers and it was done by sort of the guru of congenital, nearby ashmore goo. So what is the risk of melanoma in a congenital nevis? So the highest risk is in large and giant sized in general nearby. So anything projected to be 20 cm are greater in adulthood uh large size can general divi are also at the highest risk for extra cutaneous melanoma and also neuro cutaneous melanoma Asus which means you have melanocytes in the brain or CSF. Um Also lesions on the trunk are thought to be at the greatest risk for developing melanoma when you do get a melanoma within a general nervous, it usually occurs within the nervous itself and not in the satellites. The melanoma often presents as a new nodule in a previously sort of flat or ever so slightly raised in general nervous. And the scary part is it can occur even in the first decade of life. Um Going back to neuro cutaneous melanoma Asus, which is when you have um mole cells in the CSF or brain. Um The ones that are at the highest risk are large size or giant sized angeloni by with numerous satellite nearby, particularly if there's 20 or more um of those patients who have neuro cutaneous melanocytes most present by the age of two years, which is very early. Um and usually they have some sort of neurologic manifestations, signs of increased intracranial pressure, signs of mass lesion, spinal cord compression or things like that. And they're usually assessed through imaging such as MRI or CSF psychology. Um The majority um neuro cutaneous melanocytes are thought to be caused by somatic moses is um due to a single post I got a mutation of quote on 61 of the N. R. A. S. Jean. Um And the other thing about neuro cutaneous melanocytes patients is if you do to ever diagnose one. Those patients are also at high risk of central nervous system melanoma which has a terrible prognosis and no known effective treatment unfortunately. So how do we treat large congenital nearby? Um and this again with with all things what to do with pediatric moles and melanoma is very controversial. Um we're not even 100% certain that removing the entire lesion eliminates any risk of melanoma. Um But what we do do is get our plastic surgery, colleagues involved in san Diego. We use radio plastics who are excellent and they do plastic surgery and it often involves tissue expanders. And unfortunately if you do go ahead and do it, usually multiple stages are needed. Um We generally don't use laser has a very limited role because it usually doesn't go deep enough to get the roots of the lesion. One exciting thing though is that there was a recent publication out of Harvard um actually a graduate student's thesis on most model data on giant congenital more anesthetic nearby showing that topical administration of mexican him bitter been um I can't say that right. Ben and met Nib and seek it inhibitor imatinib actually induced nevis regression and most models which was very exciting. So maybe there's something promising on the horizon in that area. So just as a case example, this is a little girl I presented at this conference a few years ago um in this picture, she was about six months old and she was born with this large size, can general nervous on her forehead and scalp. You can see that there's multiple different colours. There's extra hair growing in it. Um So the parents were very concerned both from a medical standpoint. You know, is there any melanoma in this lesion and also from a cosmetic standpoint. Um So we ended up actually sending her to a pediatric plastic surgeon. The family selected to go to one in Chicago. Um and here she has post up on on pathology. All the results came back benign. There was no evidence of melanoma and um here's how she looked post op and from the top you can barely see anything. So I thought that was quite amazing. I wish I had a newer picture even from today. Um but consider here we would go back to the initial lesion. It's pretty amazing what what was done. The parents were very happy with what happened and you can see the scar even less nowadays, tissue expanders were needed in the surgery by the way. So, um tips for melanoma prevention. The most important thing I talk about all day long is sunscreen um in pediatrics. The American Academy of Pediatrics recommends sunscreen at age six months and above. Um and strict sun avoidance beforehand, but I always tell parents even though um sunscreen is FDA approved for ages six months and up if you are out and about no hats and your child is in the sun. I'd rather use sunscreen and let the child get a sunburn. And I recommend the ones that have zinc or titanium dioxide, um, particularly for us in California. I especially, I'm careful with the Children that are fair skinned with multiple freckles, Children with blond or red hair and a family history of melanoma. I really start educating the families early on the importance of sunscreen. One of the other things I look at is, um, whether or not there's any spider and GM is present, which are, I'll show you a picture of what those are in just a second. Those are markers of prior sun exposure. Um, and so I tell parents that there's a lot of spider in gemma's, your child probably isn't doing enough sunscreen or reapplying enough. Um, in particular in California, we have surfers, tens of swimmers, water polo lifeguards who are at higher risk of skin cancer as well. And this is a photo of the nasal bridge on a about a six year old child that's a spider angie. Um, a right there, which is caused by sun. And you can tell um, that the spider angie. Um, uh, you can, you can tell what it is by using a glass slide, putting pressure on it and it will completely blanche under Dia Skopje. Um, and those can be treated with laser cosmetically, but they're the most important thing is to educate parents that these are a marker of too much sun. Um, So in summary melanoma, the good news is it's truly very rare in the pediatric population. But if a mole doesn't look right, trust your instincts and do a biopsy s possible. Pay special attention to spits near by and large sized in general nearby and start education early about sun protection and here sunbathing dog some of you might have seen in the background and thank you so much for having me. Um I have a screaming two year old in the background so I apologize if you heard any screams from the back but he's okay. Thank you dr piggott. Um and well um forge any questions that the audiences might have. You do use these things called papoose boards and things like that to help with these young people. Well I used to and then my what I started to do what works the best is I lay the parent on their back on the exam room table. I swaddle the baby in a large warm blanket. You have to make sure that the arms and the legs are all in the swaddle and the parent actually lays on the back holding the baby. And I actually have them hook the baby in between their legs kind of like this and then I have one nurse holding the head and one assisting me and I've never had a problem, interesting way to do it. That's how we do it. It's good. Thank you Doctor Pig, you're welcome
