PHILADELPHIA, Pa. (Ivanhoe Newswire) – Thyroid cancer is the fastest growing cancer in the United States, and it's being diagnosed more often in younger patients. Previous data found the age group seeing the largest increase was white, teenage girls. But the most recent study shows it crosses all genders and races. Since this is a cancer that has few early symptoms, experts say it's especially important that parents know what to look out for.
The McCarthy kids are always moving, especially four-year old Chloe. That's why her parents never suspected something was wrong until two years ago.
“Well actually, my husband was playing with her, like you do a normal one and a half year old. Dip them upside down, bring them back, and when he pulled her up, her head was back and he saw the lump in her neck,” Chloe's mother, Lauren McCarthy told Ivanhoe.
Images showed Chloe's one thyroid gland was three times its normal size. It was cancer. Doctors removed all of the thyroid tissue.
Doctor Andrew Bauer says thyroid cancer in kids under 10 is rare, about five cases per every million, but at age 15, those numbers jump to 18 per million.
“In adolescents, it's actually quite similar to non-Hodgkin's lymphoma, which many people have heard of before,” Andrew Bauer, M.D., Pediatric Endocrinologist and Medical Director of the Thyroid Center at the Children's Hospital of Philadelphia explained to Ivanhoe.
Experts say most parents aren't aware that thyroid cancer can and does strike kids.
“They don't have any pain. They don't have weight loss,” said Dr. Bauer.
Doctors say parents should check kids for swelling or lumps in the neck. Don't skip routine check-ups, where doctors will examine neck glands.
Chloe McCarthy is cancer-free. Her parents are happy they can put this behind them.
“It's not something that we thought to look for,” McCarthy said, “It's nice to keep an eye on everything on your child.”
Doctor says with early diagnosis and treatment kids have a 95-percent chance of surviving thyroid cancer. Experts aren't sure why there has been an increase in thyroid cancers, but they say previous exposure to radiation is a risk factor.
Blood tests or ultrasound can sometimes detect changes in the thyroid, but experts say those are not recommended unless the physical exam is abnormal or a patient is at high risk, possibly because another family member has had thyroid cancer.
BACKGROUND: Thyroid cancer is the third most common solid tumor malignancy and the most common endocrine malignancy in children. The two most common types of pediatric thyroid cancer are differentiated thyroid cancer, which includes papillary and follicular thyroid cancer and their variants. Papillary thyroid cancer is the most common type in both children and adults. The second is medullary thyroid cancer in which approximately 30-percent to 35-percent are familial tumors transmitted by a gene from either the mother or father. Risk factors that can cause pediatric cancer can include occurrences in family history, radiation treatment, or radiation fallout, as discovered in increased thyroid cancer rates after the 1986 Chernobyl disaster. Although not fully understood, females tend to develop thyroid cancer more than males after puberty; the risk is equal for children, male and female, under the age of 10. (Source: http://www.entnet.org/content/pediatric-thyroid-cancer, http://www.thyca.org/pediatric/about/, http://www.everydayhealth.com/thyroid-cancer/pediatric-thyroid-cancer.aspx)
TREATMENT: Because children have a much greater chance of having thyroid cancer spread to other parts of the body, a removal of the thyroid, or total thyroidectomy, is the standard-of-care. If there is suspicion that the cancer has spread to the lymph nodes in the neck, these may be removed surgically as well. If there are any cancerous cells remaining after surgery, radioactive iodine therapy may be utilized. Thyroid hormone therapy may also be needed to replace normal hormones needed for a child's growth. These hormones include: thyroxine and triiodothyroxine, which help with growth and metabolism, and calcitonin which decreases calcium in the blood and increases it in the bones. Chemotherapy and radiation are also options depending on the type of thyroid cancer, or may be used as treatments to shrink tumors.(Source: http://www.entnet.org/content/pediatric-thyroid-cancer)
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Andrew Bauer, M.D., Pediatric Endocrinologist and Medical Director of the Thyroid Center at the Children's Hospital of Philadelphia (CHOP), talks about thyroid cancer in kids.
Interview conducted by Ivanhoe Broadcast News in September 2014.
Tell me a little bit about the increase that doctors are seeing in thyroid diseases, and specifically a thyroid cancer in children. Has there been a sharp increase over the last 5 years, 10 years?
Dr. Bauer: Over the last 10 years there's been an increase rate of diagnosis for both thyroid nodules and thyroid cancer, specifically differentiated thyroid cancer, in both adult and pediatric patients. For the majority of patients we do not reason for this increase has not been determined, however, we do not know why either develops, however, there are several risk factors that explain some of the increased rate. We know that exposure to radiation is a predisposing factor, most commonly for the treatment of a previous, non-thyroid related cancer, and we know that there are an increasing number of familial disorders where thyroid cancer can be part of the tumor predisposition syndrome. However, for the remainder of patients that do not have these risk factors, parents need to understand that there isn't anything that could have done to prevent these tumors from developing. In addition, the most important point is that no matter how a patient is diagnosed with a thyroid nodule, whether my physical exam or as an incidental finding on an unrelated radiology study of the head and neck, they need to go to a center where they can obtain a complete evaluation to determine if the thyroid nodule is a benign lesion or something that's more concerning that needs further evaluation and management.
Are you able to give me some statistics just to give us some context? Just how many pediatric cancer-thyroid cancer patients are there in the United States?
Dr. Bauer: The incidence of thyroid nodules in pediatrics has not been well studied. If you look at age distribution by the time we're 60 years old about 50% of us will have some type of thyroid lesion, a thyroid nodule a thyroid cyst. Fortunately for adults only 5-10% of these will be thyroid cancer. In addition to an age predisposition there is also a gender predisposition; females are more at risk of being diagnosed with a thyroid nodule than men are. Within pediatrics (patients < 19 years of age) the incidence of thyroid nodules is not as clearly defined. The most informative study to date looked at about 1200 head and neck ultrasound studies from patients < 19 years of age and found that 15% had a thyroid abnormality (cyst, nodule, thymic tissue…) of which 17% were thyroid nodules; a total of 9 out of 1228 studies, or 0.7%. This may not sound like a lot, however, if you consider that there are 319 million people in the United States, 27% of which are less than 20 years old, then up to 600,000 patients less than 20 years may have a thyroid nodule. In contrast, only 1 to 2% of patients will be found to have a thyroid nodule detected by physical exam. So, as I mentioned before, it turns out that no matter how you find a thyroid nodule, it is more important to determine what the nodule feels like on exam, and what it looks like on ultrasound imaging, then how the nodule was discovered in regard to deciding if the nodule should undergo fine needle aspiration biopsy (FNA). A complete and accurate evaluation when a nodule is found in a child or adolescent is critical as up to 20% of nodules may be thyroid cancer (4-5x risk compared to adults). Within this group, for both adults and pediatrics there has been some discussion of whether small, incidentally or serendipitously discovered nodules may be ‘subclinical'; a cancer that was not going to growth or spread, a cancer that the patient could have lived a full life with and died of old age rather than from the cancer. Unfortunately, it is hard to predict how the cancer is going to behave, so, we often approach care conservatively, not trying to put patients through too much as far as evaluation and treatment but also balancing that they have a long life ahead of them and treating it now may be associated with a better outcome and less anxiousness than leaving it in place. On a national basis, the National Cancer Institute (NCI) Surveillance, Epidemiology, and End Results Program (SEER) database reports that about 63,000 adults, about 130 per million adults, will be diagnosed with thyroid cancer in 2014. Within pediatrics about two to five per million children under age 10 years and up to 18 per million adolescents will be diagnosed with thyroid cancer. That sounds like a relatively small number but I try to steer away from calling this a ‘rare' cancer because it isn't rare if you're the person with the diagnosis. To put the number in context, the number of adolescent patients diagnosed with thyroid cancer is similar to the number diagnosed with non-Hodgkin and Hodgkin's lymphoma, cancers that many people have heard of before. A recent report published by the American Academy of Pediatrics reports that between 2001 to 2009 there was a 5% annual rate of increase in incidence for thyroid cancer, the cancer with the second greatest increase in the rate of diagnosis.
Give me some indication of symptoms, or what parents could watch out for?
The majority of patients are asymptomatic at the time of diagnosis, even patients where the cancer has spread to lymph nodes in the neck, which happens in about 50 to 70-percent of pediatric thyroid cancer patients. This is in contrast to other cancers where patients often have had weeks or months of not feeling well and then they discover the diagnosis as an explanation for why they felt so terrible. For both, the news is difficult, but for our thyroid cancer patients, the patients and families are a bit blind-sided and it is one of the hardest parts of the discussion; ‘how can I have cancer and still feel so well'? Even for our patients that have ‘bulky' disease, where the spread to the lymph nodes in the lateral neck can be seen on physical exam, most of the patients don't feel a lump when they're swallowing, they don't have any pain, they don't have weight loss, they don't have any symptoms, it's just that somebody a parent, another provider, a teacher, a friend or themselves discover that when they looked up or they look to the side there is a lump in the front of the neck or lymph nodes that are persistent and not going away. Swollen glands are a very common finding in children, most are secondary to an upper respiratory infection, but for patients with lymph nodes that are not going away, thyroid cancer should be considered as a potential cause.
Let's talk a little bit about prognosis. Is thyroid cancer in pediatric patients a slow growing cancer?
Dr. Bauer: What I usually try to explain to patients and their families is that the good and the bad part of (differentiated) thyroid cancer is that it is usually a slow growing cancer. This is a good thing because the slow growth is typically associated with a high likelihood of being able to surgically remove the cancer and an excellent prognosis. In fact, surgery is the most important part of treatment (and outcome) but one needs to ensure that the ‘right' surgery is performed by the ‘right surgeon. An endocrinologist that regularly manages pediatric patients with thyroid cancer serves as the ‘gatekeeper', the manager of care, ensuring accurate pre-operative staging is completed, that patients are referred to a high-volume thyroid surgeon, and that post-surgery staging and surveillance be put in place to ensure the appropriate use of medical treatment, to include if and/or when patients may benefit from radioactive iodine treatment. Regionalization of care, especially for pediatric thyroid cancer is critical to ensure that > 95% of pediatric patients with thyroid cancer go on to live happy, healthy and productive lives with the lowest risk of complications from therapy.
How do you give radioactive iodine?
Dr. Bauer: It's a pill given as a single dose. For young patients, a liquid preparation can be special ordered. The radioiodine (RAI) is absorbed by the cancer cells and the unabsorbed amount of radioactive iodine is removed through the gastrointestinal and urinary system. We use two tests to determine if a patient may benefit from receiving RAI; a blood test to look determine the level of a thyroid specific protein thyroglobulin (abbreviated Tg) and a imaging study called a diagnostic whole body scan. These tests allow us to determine if there is any thyroid cancer left after surgery and, based on the amount and location, if and how much radioiodine to administer. Two weeks prior to these tests patients stop their thyroid hormone replacement and start a special low-iodine diet in order to optimize the evaluation and potential treatment. RAI is colorless and odorless and is cleared from the body over 2 to 5 days after a dose. So patients and family members are educated and provided directions in order to increase clearance from non-thyroid tissue and to avoid exposure to other family members. Patients are considered for repeat RAI if there is evidence of persistent disease that is growing and cannot be removed surgically. This is usually 12 or more months after a previous RAI dose. This is the touch part of treatment that I mentioned earlier, the ‘bad' or difficult part is to be patient in an effort to determine if and/or when additional therapy is needed even when the blood test (Tg) suggests that all the cancer many not have been eliminated. Our patients and families need to be lifelong partners with us and to be patient. We wait as long as possible between treatments in an effort to see how much we get from each surgery and/or dose of RAI. People want immediate resolution of the cancer, to achieve cure as fast as possible. This may happen in 3 months for some, but 18 months or longer for others. For a cancer that grows slowly, and is not associated with high mortality, we work to balance and individualize how and/when additional treatment would be beneficial.
You mentioned that you are not sure why children and adolescents develop this kind of cancer, why it seems to happen more in adolescent girls, and why the increase in the number of children being diagnosed; could it be hormone related?
Dr. Bauer: That's a reasonable guess and a research area of interest that I have as far as trying to figure out why females are diagnosed more than men and girls more than boys in the pediatric age. Saying that estrogen must be related seems to be pretty obvious but the reality is that we don't have the data to say that estrogen is an absolute and/or how it may be related to allowing a cell to become cancerous and behave in regard to treatment. As an example, there are data that thyroid cancer is more aggressive, less responsive to therapy, in older patients and in men. So, we really need to increase how much time and money we are investing into figuring out why thyroid cancer develops during the pediatric age, if there are things we could change in our environment to decrease the risk of it developing it, and to find markers to predict how the cancer will behave and respond to therapy in an individual patient.
Is there anything I didn't ask you that you think is important to know?
Dr. Bauer: I think we've touched on a lot of the important aspects. The only other recent item that is of interest is the recent movie that is based on two teenagers with cancer, the boy with bone cancer and the girl with thyroid cancer that spread to her lungs.
Is the movie increasing awareness for pediatric thyroid cancer?
Dr. Bauer: I haven't seen the movie yet so I'm at a disadvantage, but my daughter and several of our patients that have seen it describe that it is based on a patient that had thyroid cancer and then developed ‘lung cancer'. The reality is that the movie is based on the true story of a strong, independent and very engaging girl that had papillary thyroid cancer that had spread to her lungs, a site for metastases that happens in about 15 percent of our patients that have significant spread to lymph nodes in the neck. So, it is not ‘lung cancer' it's thyroid cancer in the lungs. For the majority of pediatric patients when the cancer spreads to the lungs it continues to absorb iodine so that we can use radioactive iodine to treat the cancer. About 20-30% of patients develop stable lung disease that does not grow but we can't get rid of it, and less than 5% develop disease that no longer absorbs iodine. So, overall, even for patients with metastasis to their lungs, the long-term outcome is excellent. Sadly, Esther (Grace Earl), the patient that the movie is based on, developed progressive lung metastasis that did not absorb radioactive iodine and despite excellent care and amazing strength it continued to grow and she died from thyroid cancer. So, the wonderful part of the movie is that it is based on the life of someone all of us would be proud and humbled to have known and cared for. The difficult aspect of the movie is that it reminds us that cancer is cancer and we don't have all of the answers. The movie helps raise awareness that there are no good cancers, that even patients that enter remission after one surgery and one treatment of RAI still had cancer and we have an obligation to figure out why it developed and what we can do to improve diagnosis, improve our ability to predict prognosis, and to ensure patients are cared for in centers with expertise in an effort to reduce complications.
Have you found that with establishing a thyroid center, how it helps to have that expertise when you're dealing with something like this? You mentioned it's a partnership.
Dr. Bauer: Having the Children's Hospital of Philadelphia invest in creating a thyroid center has been very rewarding. The center includes about 7 different divisions but with only 1-2 providers from each division. This affords improved communication and improved care, from diagnosis to treatment. To provide appropriate care, you need expert endocrinology, oncology, surgical care, radiology, pathology, ophthalmology and behavioral health all in the setting of a pediatric hospital. The endocrinologist has to be able to identify patients at risk, to perform an accurate exam, and to review the ultrasound images in order to decide who may need to have a biopsy. You need to set up a system where the biopsy is performed in an environment where the patient is comfortable and that the likelihood of an adequate sample and accurate diagnosis is ensured. You have to have a pathology that can examine the cells and decide if they look like cancer cells or whether they look benign (not cancer). You need to have 1 to 2 surgeons that perform thyroid surgery regularly to ensure that as much of the cancer is removed as possible with the least risk of complications. Ideally, thyroid surgery should only be performed by a surgeon that is performing 30 or more thyroid surgeries per year. After surgery, your pathologist needs to determine what type of cancer it was, whether papillary thyroid cancer (the most common), follicular thyroid cancer, their variants or medullary thyroid cancer. Then the endocrinologist has to work with the nuclear medicine physician to decide if RAI would be beneficial, and, last the endocrinologist also needs to manage the surveillance plan to determine when a patient is in remission, to follow normal physical growth and development, and to decide if and/or when additional treatment is necessary. Oncology is a nice addition to help screen high-risk populations and to help manage patients that develop disease that may benefit from targeted chemotherapy that is not responding to the standard approach to care. So, the advantage of having a thyroid center that is organized around this structure, that ensures regular communication between team members across divisions, cannot be over stated or overemphasized. In addition to our monthly tumor board we are in constant communication. Our nurse coordinators ensure a single path to access care and to ensure timely response to calls and we arrange our endocrine clinic to coincide with the clinics of our two surgeons, Dr. N. Scott Adzick and Dr. Ken Kazahaya. For patients that travel from out of town, we can coordinate evaluation and surgical treatment for the same week. Most endocrinologists have experience with medical treatment of Graves' disease (hyperthyroidism), congenital hypothyroidism and acquired hypothyroidism. But, with a higher volume, and providers that have been afforded an opportunity to focus their clinic time and research on thyroid disease, the center provides greater expertise to evaluate and manage typical thyroid disorders and to determine and care for patients that don't follow the plan, don't follow the what our textbooks describe as the usual course.
About the patient we're going to see tonight can you tell us about her condition? Is she four years old?
That's really young isn't it? I mean teenagers are young to me too, but to have somebody in that age, under ten years?
Dr. Bauer: Yes, you're right. For children that are prepubertal, and even preschool age, the likelihood of having the most common thyroid disorder, congenital hypothyroidism is about one in 2,000. Every State in the United States screens newborns for this disorder. However, this is the least common age group for patients to be diagnosed with thyroid nodules and thyroid cancer. There are some tumor syndromes that can present in this age group, the most common being multiple endocrine neoplasia type 2 (MEN 2) where there is a very high risk of developing a form of thyroid cancer that does not absorb radioiodine called medullary thyroid cancer (MTC). There are also some patients that are referred for evaluation of a thyroid nodule and it ends up that they have a variation of development where a piece of thymic tissue is located within their thyroid gland, in addition to the remainder of the thymic gland being in the chest cavity, the normal location. But, although less common, patients in this age can develop true thyroid nodules and thyroid cancer, at times cancer that is very invasive. Chloe had follicular thyroid cancer (FTC) which is another form of differentiated thyroid cancer. It is much less common across all ages and within a prepubertal child it's really very uncommon. She had a large nodule, four centimeters in size that was initially discovered by her pediatrician. It was a good pick up by someone doing a thorough exam. Her initial surgery was not performed at CHOP and was limited to only removing ½ of the thyroid gland. When the family came to us the question they asked was what should we do now that we've found thyroid cancer, should we have another surgery to remove the remaining thyroid tissue and does she need reactive iodine? We talked to the family and decided that a completion thyroidectomy, doing the second surgery, was important as it would allow use to use the lab tests, the thyroglobulin (Tg) level to follow her and then decide if radioactive iodine may be of benefit. So, while none of us were excited about the second surgery, we were comforted that the complication rate was low as one of our two thyroid surgeons was performing the surgery in a pediatric hospital with the expertise to care for a 4yr undergoing surgery. Dr. Adzick completed the surgery and performed an oncologic thyroidectomy, removing all of the remaining thyroid tissue. Her Tg level post-surgery has been undetectable, so, as of now, it appears that we have achieved surgical remission without needing to use radioactive iodine, a huge benefit to Chloe. So, that's one example of the advantages of going to someplace that sees patient with less common disease more frequently and another reason to support creating regional centers for care. It's the only way you can increase the expertise and optimize caring for patients with less common diseases. As far as other patients Chloe's age, we care for patients with all forms of thyroid disease, from congenital hypothyroidism, to hyperthyroidism, thymic tissue in the thyroid, thyroid nodules, and several patients with thyroid cancer that has spread to lymph nodes in the neck and to their lungs. Less common than in adults, less common than in teenagers, but, it really does happen in this young age group, and it reinforces the advantage of having a pediatric thyroid center.
With the statistics we talked about, the 5 per million under age 10, 15 to 18 per million in 15-18 year olds, is that a specific type?
Dr. Bauer: Yes, these numbers refer to differentiated thyroid cancer, to include papillary and follicular; papillary thyroid carcinoma (PTC) is about 90 percent of the cases, follicular thyroid carcinoma (FTC) is about 5%, and the rest mixed PTC and FTC, or, medullary thyroid carcinoma (MTC).
Follicular is the most common?
Dr. Bauer: No, Papillary thyroid carcinoma, PTC, is the most common.
Are those are the most recent statistics across all pediatric ages?
Dr. Bauer: Yes, the most recent data is from the NCI SEER database is through 2011 and the numbers above cover the least to the most frequent in patients < 19 years of age.
When you say 2 to 5 per million children under age 10 years is that an increase or just a range? Do you know what the statistics were 10 years ago?
Dr. Bauer: Within the prepubertal that incidence has been pretty stable. It's just within the adolescents age group that the rate has gone up from let's say 8 to 10 per million to about 18 per million over the last two decades. This data is only referring to patients with a confirmed diagnosis of thyroid cancer, the number of patients with thyroid nodules has increased well beyond that, but outside of the data I mentioned earlier in the interview there isn't a national registry to track pediatric patients with a diagnosis of a thyroid nodule. This is another limitation of having patients undergo evaluation at non-thyroid center, there is less that is known about the number of patients undergoing evaluation. We continue to see patients that were referred from their primary provider directly to a local ENT or surgeon and the patient diagnosis, typically thyroid cancer, is never entered into the State reporting system that is mandated at all pediatric tertiary care hospitals. Endocrinology, both adult and pediatric, should be the first place a patient is referred if a nodule is found on exam. They are in the best position to ensure an adequate and appropriate evaluation is completed and to ensure that if a patient would benefit from surgery, that the patient is sent to a high-volume thyroid surgeon. Having fewer, but regional centers of care, is the ideal path to ensuring stratification and individualization of care, of decreasing complications and for setting up a system to better track the incidence of a disease as well as outcomes of therapy.
This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.