Stacy Willingham

When Arianne Missimer, a doctor of physical therapy, a dietitian and the founder of the Pennsylvania-based integrated health center The Movement Paradigm, discovered a mass behind her right knee after feeling nerve pain in her leg, she quickly scheduled an ultrasound.

“The pain wasn’t anything that was really limiting me,” Missimer says. “It was just an unusual symptom. If I didn’t know my body so well, it was something I could have easily ignored.”

After the ultrasound confirmed the mass was solid, she underwent an MRI. The results were startling. In March 2015, at age 34, Missimer received a diagnosis of stage 3 liposarcoma, a rare type of cancer that develops in the fatty tissue.

“I remember looking up ‘sarcoma’ and seeing that there are so many different types,” she says. “Then I looked up ‘liposarcoma,’... but there wasn’t a ton of information out there.”

This isn’t that uncommon, according to Dr. Melissa Burgess, a medical oncologist at UPMC Hillman Cancer Center in Pittsburgh. “Unfortunately, for some of these diseases, there is no clear standard next step,” she explains. “There’s a lack of data because it’s a difficult disease to treat. It’s also rare, so it’s hard to do large studies.”

“Sarcoma” is the general term for a broad group of cancers in the bones and connective tissues. Out of the approximately 1.9 million people who received a cancer diagnosis in the United States in 2020, only 17,000 — less than 1% — had a sarcoma, according to Dr. Robert Maki, a medical oncologist and professor of medicine at the University of Pennsylvania Perelman School of Medicine in Philadelphia.

There are two general categories of sarcoma. Soft-tissue sarcomas affect tissues that connect, support or surround the organs and include sarcomas that are present in the muscles, tendons, fat, blood vessels, nerves, fibrous tissues and synovial tissues. Bone sarcomas grow in the bone or cartilage. Between these two categories, sarcomas span 70 subtypes, often making a precise diagnosis difficult.

Brenda Kennedy, a yoga instructor and personal trainer in El Paso, Texas, experienced this difficulty after visiting the doctor for a pain that she describes as a “dull toothache in the knee.” After undergoing an X-ray, a bone scan, a PET scan and an MRI, she was found to have a grade 2 chondrosarcoma in 2009 at age 31.

“But the most frustrating thing was how long the imaging took. I couldn’t get a referral to an orthopedic oncologist until they ruled out everything else,” Kennedy says.

“In terms of the frequency, about 3,000 people in the United States develop liposarcoma each year; for chondrosarcoma, it’s around 1,000 people a year,” Maki says. “But even the terms ‘liposarcoma’ (and) ‘chondrosarcoma’ represent a family of tumors with further subtypes.”

Liposarcoma subtypes include well differentiated/ dedifferentiated, myxoid/round cell and pleomorphic. For chondrosarcoma, approximately 90% are conventional chondrosarcomas, and the other 10% are subtypes, which can include dedifferentiated, clear cell, mesenchymal and myxoid.

In addition to being both rare and highly specific with their subtypes, liposarcoma and chondrosarcoma also have few risk factors and often-vague symptoms, increasing the difficulty and timeliness of diagnosis. Maki notes that certain genetic syndromes, exposure to radiation for treat- ment of another cancer, exposure to certain chemicals or a damaged lymph system could increase a person’s risk for a sarcoma. For chondrosarcoma specifically, a family history of developing multiple benign cartilage tumors is one factor that could increase a person’s risk.

“We consider them bad luck tumors,” Maki explains. “There’s nothing people did to get them. There’s no association with eating, drinking, smoking ... nothing like that.”

“Over 80% of these cases are spontaneous,” Burgess emphasizes. “That’s what makes it so challenging. We don’t know what causes them, we can’t really screen for them and they are so rare.”

Both Missimer and Kennedy led healthy lives as athletes prior to their diagnoses. Other than the leg pain that brought them both to the doctor, neither of them exhibited any serious symptoms.

“I remember being so angry because I had done everything right,” Kennedy says. “They say, ‘You need to eat right. You need to exercise.’ I taught fitness classes. ... I did everything you are supposed to do, and I was (still) dealing with a cancer diagnosis.”

Liposarcoma is most common in adults ages 40 to 60 and presents in the extremities (60%), abdomen (10%), and head and neck (10%). Chondrosarcoma, most common in adults who are 30 to 70 years old, can present in any part of the skeleton. When it comes to symptoms, the type and severity can largely depend on where the tumor is located as well as the stage and grade.

“Liposarcomas mostly show up as a painless lump, like most cancers,” says Dr. Atrayee Basu-Mallick, director of Sidney Kimmel Cancer Center’s Multidisciplinary Bone and Sarcoma Center. “That is why in certain locations — for example, within the abdomen — diagnosis can be delayed. Symptoms happen only when the tumor is pressing on another organ, nerves or blood vessels.”

For Missimer, her pinpoint nerve pain likely occurred once the tumor grew large enough to press against her sciatic nerve. She noticed it most when sitting down and crossing her legs. A majority of patients may have a similar experience, but others could experience symptoms earlier.

Liposarcoma in the head and neck, for example, usually causes a noticeable fullness; in the abdomen, one may experience difficulty eating and going to the bathroom and swelling or distention due to growth of the tumor. As with most other cancers, it can also present with nonspecific symptoms, such as weight loss, chills, fever, fatigue and night sweats. For some, the tumor never exhibits symptoms at all and is found incidentally.

Chondrosarcoma symptoms, on the other hand, usually appear before a lump is noticed.

“(These) can include a sense of pain, stiffness and a change in the range of motion in the joints close to where the tumor (is) growing,” Basu-Mallick explains. “If you have worsening symptoms, swelling or a lump you can feel, it is always good to get it evaluated.”

According to Basu-Mallick, the best course of treatment for each patient typically depends on five factors: exact type of tumor or histology, grade, stage, location and the patient’s overall clinical picture, which is determined by any other health issues.

“For sarcomas, ‘grade’ is the most important factor for long-term prognosis,” she says.

Grade refers to how the tumor cells look under the micro- scope, whereas stage refers to the tumor size, and local and distant spread. To determine grade, factors such as how many cells are actively dividing, the number of cells that are dead and how the cells look in general compared to the primary tissue of origin are all taken into consideration.

In a grade 1 tumor, for example, a patient could have surgery for removal. However, for grades 2 and 3, treatment gets more complicated — sometimes including a combination of chemotherapy and radiation — and the surgery is often expanded to remove areas around the tumor itself.

“Sarcomas are like a mound of salt,” Basu-Mallick says. “The mound is the tumor, but there are little grains all around it.

We have to get that microscopic disease out ... so to be safe, we have to take out a bigger section.”

When putting together a treatment plan, Burgess, Maki and Basu-Mallick agree that taking a multidisciplinary approach is best.

“I always preach that it’s helpful to have your case reviewed by multiple doctors in different disciplines who understand the disease and can help come up with the plan,” Burgess says. “Getting to a center that has experience in sarcomas is also really important.”

For Kennedy, treatment involved removing 15 centimeters of her femur and inserting a metal allograft. She then underwent three more surgeries — including a total knee replacement, megaprosthesis and, due to her active lifestyle, repairing cracks in the polycarbonate structure — over the course of five years. Because she had relocated, Kennedy had surgery at different locations.

“Movement and being outside has always been my form of relieving stress,” Kennedy says. “I was dealing with the most stressful thing in my entire life, and my one way of coping with it had been taken away from me.”

Missimer, on the other hand, underwent fertility preservation, six rounds of chemotherapy, radiation, proton therapy and a limb-sparing surgery that removed the tumor and part of her hamstring. “I felt really good during the proton therapy, mentally, emotionally and physically,” says Missimer, who started training for American Ninja Warrior, a sports entertainment competition based on tough obstacle courses, while
still in treatment. “Then I got the surgery, and that was emotionally tough.”

Despite the complications that rare cancers such as these can bring, medical professionals are optimistic about where the future is headed.

“Since these tumors represent multiple types of cancers, there’s an interest in breaking each one down and finding specific treatments,” Maki explains. “For example, half of regular chondrosarcomas have a mutation in a gene called IDH1 or IDH2, which lead to hyperactivation of the encoded enzyme isocitrate dehydrogenase, and there are now drugs that target those mutations. There are hints of benefit for people who receive those medications, though it’s still very early in the development of those drugs.”

Maki also notes a clinical trial that is looking at a different way to trigger the death of the chondrosarcoma cell without having to use chemotherapy.

“The big problem with chemo for chondrosarcoma is that chondrosarcoma is in the cartilage, where there are no blood vessels. You can’t just inject a drug into the veins and expect it to get to the tumor,” he says. “But by using a monoclonal antibody that sticks around the body for weeks, you can achieve a level of medication in the body to kill off those tumor cells.”

Finally, although sarcomas are broadly thought to be a cancer that haven’t proved responsive to immunotherapy, oncologists including Burgess are dedicating their time to looking at certain genes that are expressed in the tumor and then identifying unique characteristics within subtypes that will be responsive to immune treatments.

Today, both Missimer and Kennedy are cancer-free. Kennedy’s fourth and final surgery took place in March 2014, and she has maintained her active lifestyle by walking three half-marathons, teaching yoga, camping and keeping up with her four kids. As for Missimer, three months after her surgery, she went on to compete on “American Ninja Warrior” in May 2016.

“Physically, I have limitations, but I was able to manage my pain and do my physical therapy, and I attribute that to now being as functional as I am,” Kennedy says. “I would tell patients to mentally prepare for pain; mentally prepare for keeping up with the physical therapy and be as active as you can, within reason, to keep your body going.”

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Articles

Despite the challenges of diagnosing and treating liposarcoma and chondrosarcoma, experts are optimistic about future treatments for these two rare cancers.

Navigating the Unknown of Rare Cancers

In April of 2019, Colt Blunt arrived to a routine physical prepared with notes describing some gastrointestinal symptoms he had been experiencing, including heartburn, bloating, abdominal pain and general discomfort.

“My symptoms weren’t that bad, but they were new,” says Blunt, a forensic psychologist in Minnesota. “Luckily, I have a fantastic primary care doctor, so he took me seriously. He said it was probably just acid reflux, put me on some proton pump inhibitors and said if I wasn’t perfectly back to normal after 10 days, he would send me for an endoscopy.”

Although the medication helped, it didn’t eradicate Blunt’s symptoms, so the following week, he went in for an endoscopy. At 37 years old, Blunt was young and otherwise healthy. However, because his family has a history of cancer, when the endoscopy visualized an ulcerated area of his cardia, he was concerned about the risk.

“I asked my (gastrointestinal) doctor ‘What are the chances that this is cancer?’ and he said, ‘One in a million,’ “ Blunt says. “When I asked him why, he said that he had just diagnosed a 37-year-old with stomach cancer the week before, and he sure as hell wasn’t going to diagnose another one a week later.”

However, on June 20, 2019, that’s exactly what happened. After his endoscopy, Blunt received a diagnosis of stage 3 gastric adenocarcinoma of the upper cardia (just below the esophagus). Although gastric cancer at Blunt’s age is rare, his experience is representative of a growing trend — an increasing number of patients under the age of 50 are receiving a diagnosis of gastrointestinal cancer.

“I used to always be younger than all of my patients, and now I’m seeing patients younger than myself,” says Dr. Travis Grotz, a surgical oncologist in the Division of Hepatobiliary and Pancreatic Surgery at Mayo Clinic in Rochester, Minnesota. “It is crushing when you see a young mother or father dealing with something so serious.”

“Gastrointestinal (GI) cancer” is a term used for the group of cancers that affect the gastrointestinal tract and other organs contained within the digestive system, including the colon, rectum, liver, stomach, pancreas and esophagus. As with many cancers, GI cancer mostly affects older popul- tions. For example, the average age of people who receive a stomach cancer diagnosis is 68; for colon cancer, the average age at diagnosis is 68 for men and 72 for women. Why, then, is incidence for GI cancers rising in younger patients?

Rebecca Siegel, senior scientific director at the American Cancer Society, first set out to answer that question, in regards to colorectal cancer, in 2007.

“I was analyzing data from a colorectal cancer report, and for some reason, I decided to analyze trends by age,” Siegel says. “In the oldest age groups, incidence and mortality (are) declining rapidly and (have been declining) for a long time now. ... But when I looked at rates in adults under 50, I noticed that incidence was actually increasing — and by a statistically significant amount.”

What’s more, Siegel clustered that data from adults under 50 into several smaller age groups — 20 to 29, 30 to 39 and 40 to 49 — and found that incidence rates were increasing across all of them. Similar studies are taking place across other GI cancers, too, with very similar findings.

One study was co-authored by Dr. Don Codipilly, a gastroenterology fellow at Mayo Clinic, in January. When analyzing data from patients with esophageal adenocarcinoma between 1975 and 2015, Codipilly and colleagues found that not only has esophageal adenocarcinoma incidence increased in patients under age 50, with an annual change of 2.9%, and presented at more advanced stages than older patients, but younger patients with esophageal adenocarcinoma also experienced poorer five- year disease-free survival (the length of time after primary treatment for a cancer ends that the patient survives without any signs or symptoms of that cancer) compared with older patients.

“Obviously, this is an unsettling trend because with cancer, we would hope that incidence would be going down,” Codipilly says.

Because GI cancers are more prevalent in older populations, limited data are available on younger patients, making it difficult to ascertain why incidence is rising among people under the age of 50.

“There are known risk factors for colorectal cancer, such as excess body weight and smoking, but all of those studies are based on people who are much older,” Siegel says. “All of this is a really new area, and (increasing incidence in younger populations) doesn’t seem to be fully explained by those risk factors that we currently know of.”

For example, Siegel says that they are not seeing the same increase among racial and ethnic groups; the largest increase is in non-Hispanic White patients, which suggests that it isn’t just excess body weight contributing to the rise, because obesity has been increasing in all of these groups. The trends are also “remarkably similar” between men and women, which suggests that it is not hormonal.

However, researchers are learning more by the day, such as the role gut bacteria may play in the development of certain cancers.

“I have been very impressed with the increasing literature suggesting that the bacteria in our GI tract may play important roles in the development of cancer,” explains Grotz. “This may be particularly true in young patients. As a result, this has widespread implications of how we manage antibiotics.”

Even with an increasing number of younger patients receiving a diagnosis of GI cancers at more advanced stages, obstacles to getting that diagnosis still remains.

“If a young person who is 20 or 30 (years old) goes to their doctor with a GI complaint, cancer is not coming to their or the physician’s mind,” notes Codipilly.

This is exactly what happened when Matt Budgell first started experiencing chronic stomach pain in February 2019.

“I wasn’t getting enough hours at work to get health insurance through my employer, so I just started getting used to dealing with the discomfort. Cancer was the furthest thing from my mind,” says Budgell, who was 26 years old at the onset of his symptoms. “When I finally got in to see a gastric specialist, they said, ‘You’re young. You probably just have an abnormal amount of acid or an ulcer in your stomach. Take some Prilosec OTC and you’ll be good to go.’ I tried that, and it didn’t do very much at all.”

After having his endoscopy appointment continually rescheduled — once, Budgell says, because they gave his appointment to somebody else who “needed to be seen right away” — he was finally given answers on Jan. 10, 2020, almost a year after his symptoms began. At age 27, he was told he had stage 3/4 gastric cancer.

“There is no doubt in my mind that my age played a role in why it was so hard for me to get an appointment,” Budgell, who initially received treatment in his home state of Hawaii and later California, says. “They push young people off to the side. They expect you to be fine. And then all this time passes, and finally, you find out that you’re not going to be fine at all.”

Siegel highlights that later diagnoses like that of Budgell play a critical role in why cancers in younger patients are more advanced — so, as with any cancer, early detection is important. However, because younger patients tend to be healthier than those over 50 years of age and are less likely to have comorbidities, they can also handle more aggressive treatments.

“A lot of our current treatment regimens (for GI cancer) are targeted at an older population, but younger people can probably withstand more aggressive treatment or major surgeries,” Codipilly says. “That could lead to more complications, but it could also lead to greater rates of cure.”

Immediately after getting his diagnosis, Budgell had an open partial gastrectomy to remove the tumor, 60% of his stomach and a piece of his pancreas, followed by four months of chemotherapy. Blunt, too, underwent an aggressive treatment comprised of an open full gastrectomy (a complete removal of the stomach) and eight rounds of chemotherapy.

“I didn’t want (a doctor) who was going to treat me like I was medically fragile,” Blunt says. “I wanted an oncologist and a surgeon who were willing to look at me as an individual, not as a group statistic.”

For both of them, despite the advanced stage of their cancers, the aggressive approach paid off. Budgell’s most recent follow-up PET scan and endoscopy looked clear, and Blunt has been NED, or no evidence of disease, on all of his scans since August 2019. Now the biggest hurdle involves finding the best way forward — changing their diets and dealing with psychological after effects.

“My diet has been completely overhauled. I used to eat whatever I wanted and never gained any weight, so it felt like it didn’t matter, but after this experience, I eat really clean,” explains Budgell. “For me, the recovery from the surgery was not as bad as the psychological damage and recovery that had to take place.”

“It took me almost a full year to figure out how to eat enough to get all my calories in,” notes Blunt. “I can’t eat dairy and sugar like I used to; meals, in general, have to be smaller than they used to be ... but I’m back in the gym, back to running, back to working full time, and I’m feeling pretty healthy. I’m still learning things about my new normal, but anybody looking at my life from the outside who doesn’t know what I’ve gone through probably thinks I’m living a pretty normal life.”

However, although young individuals may be better able to withstand treatment, as well as bounce back quicker after treatment, their age can also come with some disadvantages.

“Young folks are not usually as financially stable as older patients,” Siegel says. “They often have young families to care for and are in the beginning or middle of their career, and a lot of drugs they are receiving are very expensive with high out-of-pocket costs.”

“Being a young adult and not dealing with cancer, it is already hard enough to get by and make ends meet,” Budgell says. “But when you’re diagnosed, you have to think about treatment and taking time off because you don’t feel well, (but) your expenses don’t just go away even though you’re sidelined by cancer.”

Experiences like those shared by Blunt and Budgell underscore the unique challenges young patients with GI cancer face, as well as the importance of both patients and doctors taking all symptoms seriously, despite the age of the patient.

To get ahead of a potentially serious diagnosis, Grotz suggests, patients should have health insurance whenever possible and bring up every symptom to their doctor, no matter how benign that symptom may seem. If patients feel that their doctors are discounting their concerns, they should advocate for themselves and find a new doctor, if necessary. They can even empower themselves with knowledge and point out the statistics of rising cases of GI cancers, as well as others, among young individuals.

“All of us should be going to the doctor at least once a year,” Blunt says. “Listen to your body, and if something feels off, talk to somebody about it. If you have a doctor that brushes you off and says it’s nothing, then that’s a bad doctor — get a new one. If I had a different doctor who didn’t take me seriously, I would probably be dead. It would have been caught too late.”

Siegel also stresses the importance of knowing your family history and relaying that information to your doctor.

“There are a lot of places to fall through the cracks, but knowing your family history is a low- hanging fruit,” she says. “We could make a huge impact if everybody knew their family history and was screened according to guidelines.” Although there are no strict screening guide- lines for many GI cancers, Siegel’s research contributed to the American Cancer Society lowering its recommended age to start screening for colorectal cancer — from age 50 to 45. “Reducing the age recommendations is huge,” Siegel says. “However, while colonoscopy is the most common test, for even younger people ... there are home tests that are very effective. The best test for you is the test that you do.”

A cancer diagnosis can be isolating, no matter your age, stage or cancer type. And because gastrointestinal (GI) cancer typically affects older populations, receiving a diagnosis as a young adult comes with a unique set of challenges.

“A lot of times, folks with cancer can feel like they’re doing it on their own,” explains Dr. Don Codipilly. “But there are tons of people out there who have gone through similar issues.”

For that reason, patients should never underestimate the importance of finding a community of other patients with cancer and survivors who understand what they’re going through. Codipilly suggests starting by asking a doctor to recommend local support groups; if there aren’t any available locally, many national groups are able to connect patients from across the world over the internet.

Both Colt Blunt and Matt Budgell, two young adults with GI cancer, have been involved with Debbie’s Dream Foundation since receiving their diagnoses. The nonprofit organization is dedicated to raising awareness about stomach cancer, funding research and providing support to patients, families and caregivers.

“I became involved with Debbie’s Dream Foundation after searching for cancer survivor stories on Google shortly after my diagnosis,” Blunt says. “Through Debbie’s Dream Foundation, I’ve met other survivors who I’ve been in frequent contact with since.”

Since finding the foundation, Blunt has also started advocating and mentoring, as well as helping to open a local chapter of Debbie’s Dream Foundation in his home state of Minnesota.

Budgell took his advocacy a step further by founding his own nonprofit, The Love for Life Foundation.

“I didn’t connect with any other young adults with cancer until after my treatment was over,” notes Budgell. “After I started the foundation, I started realizing how much of a benefit that human connection is after the cancer experience, so I can only imagine the impact it would have made on me during the experience itself.”

Budgell describes the foundation as his way of “letting other young adult cancer patients and their partners, caretakers and families know that they are not alone on their journey.”

In addition to providing resource navigation, mentorship and human connection to young patients, The Love for Life Foundation provides rent relief funds for qualified applicants.

“I was making $300 a week on disability (during my treatment), and that was not enough to make it,” Budgell says. “If it (weren’t) for the support we got from GoFundMe and other cancer nonprofits, we would have been in real trouble. I was blessed with the support I received, and I felt like it was my responsibility to pay it forward ... and create an extension of that same love and support for others.”

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As the incidence of GI cancer creeps up among younger patients, so does the question of why.

Hard to Stomach: The Rise of Gastrointestinal Cancers in Patients Under 50

As she walked into Memorial Sloan Kettering Cancer Center, Erin Hazelton was struck by the appearance of the woman in front of her. Hazelton was at the New York, New York, cancer center to begin treatment for stage 2 invasive ductal carcinoma.

“I walked into the center right after another woman, and when I heard her give her date of birth (to the receptionist), I realized that she was only a couple of years older than me, but she looked like she was my mother’s age,” recalled Hazelton, who received her diagnosis in 2018. “When you (get a diagnosis) at age 37 (like I did), it’s scary. So much of my identity was tied up in how I looked. I was terrified.”

Although many cancers are unseen, different types of treatment can lead to painful, visible side effects. After starting treatment, Hazelton experienced skin side effects including universal hair loss; facial wrinkles and loss of collagen; melasma (dark/discolored patches) on her face; dry skin; seeping wounds; scars; and radiation tattoos.

“In addition to the regular side effects of chemotherapy, which can be devastating to quality of life, newer targeted therapies and immunotherapies have additional side effects of the skin that can be very frequent and very specific,” says Dr. Nicole LeBoeuf, a dermatologist at Brigham and Women’s Hospital and director of the skin toxicities program at Dana- Farber Cancer Institute in Boston, Massachusetts.

According to LeBoeuf, systemic cancer treatments can generally be grouped into three categories, and the types of side effects patients could experience will depend on the patient’s treatment regimen and cancer type.

The first category is cytotoxic, or classic, chemotherapy, which most commonly causes side effects including alopecia, or hair loss; painful rashes on the hands and feet; and general rashes elsewhere on the body that can lead to swelling, pain and itchiness.

Hazelton’s initial regimen included Adriamycin (doxorubicin), a chemotherapy drug known as “the red devil” because of its harsh side effects, followed up with Taxol (paclitaxel). “I lost all my hair — eyelashes, eyebrows, everything — and my skin looked like I had aged a good 15 to 20 years toward the end of it,” Hazelton says. “My cheeks had wrinkles because the collagen wasn’t being renewed; I had melasma, which I hadn’t expected, that was made worse by the sun.”

She also experienced skin-related side effects from radiation and scars from her lumpectomy. “My skin started slowly breaking down over the course of my radiation, and at the end of six weeks, I had seeping wounds,” Hazelton says. “My nipple looked like it was going to separate from my body.”

The second category of treatment that can lead to skin side effects are targeted therapies, which are linked to the specific drug used for that patient’s treatment. “The most commonly talked-about skin side effect of targeted therapy is called a papulopustular, or an acne-like, rash from EGFR inhibitors, or drugs that target the epidermal growth factor receptor mutation. This mutation is found in lung cancers, head and neck cancers, some colon cancers and, rarely, in breast cancer,” LeBoeuf says. “In fact, 85% of patients who are treated with drugs that target that particular genetic mutation will develop that rash.”

Another common skin side effect from targeted therapies is hand-foot skin reaction, which causes painful calluses and blisters on pressure points. “These blisters can occur from doing very normal activities,” LeBoeuf says. “Something as simple as going to the grocery store can make a patient’s feet look like they’ve just run a marathon. Someone who works on a keyboard may experience painful blisters on their fingertips when they type.”

Severe skin side effects such as these can prevent patients from resuming everyday activities and make them more likely to discontinue their cancer treatment, leading to a worse prognosis.

The third category of treatment leading to skin side effects is immunotherapy, a type of treatment that boosts the body’s natural defenses, or immune system, to fight the cancer. “Immunotherapy is a beast because once you unleash the immune system, you can turn on any and all skin diseases that would happen outside the cancer setting. Anything that could possibly happen in the field of dermatology has been triggered by activating the immune system,” LeBoeuf says. “It is amazing and it is groundbreaking...but it can also lead to autoimmune disease in any organ.”

The most common skin-related side effects of immunotherapies include psoriasis, with bumpy red patches covered in white scales; vitiligo, or whitish patches from lost pigment; and lichen planus, an autoimmune disease that can cause swelling and irritation of the skin, hair, nails and mucous membranes.

Sometimes the adverse immune events that are activated through immunotherapy persist after the treatment has ended. “Our approach to these side effects is always to try to uncouple the toxicity from the effects of the drug on the cancer, then target the side effects as specifically as possible,” LeBoeuf says. “This leaves the rest of the immune system intact to fight the cancer.”

Given the variety of possible side effects and the degree to which it may affect a given individual, it can be hard for patients to know which ones to expect and how to manage them if they arise during and after treatment.

Dr. Anisha Patel, an associate professor of dermatology at The University of Texas MD Anderson Cancer Center in Houston, recommends that patients follow good hair, skin and nail habits before starting treatment: “Moisturize often, avoid perfumed products, and decrease the use of lacquer on your nails,” she says.

Patients who have a history of skin conditions prior to their cancer diagnosis are more likely to see worsening during treatment. “If you already have eczema, psoriasis, or acne, those things are more likely to be exaggerated,” Patel says. “If you have a preexisting skin condition, that should be taken care of before your therapy starts, as well.”

During treatment, certain practices can help prevent the most common side effects. For example, wearing ice- cold gloves and socks and using scalp cooling treatments to restrict blood vessels reduces the chances of hair loss and hand-foot syndrome.

Patients should also take steps at home to minimize side effects. Wearing sunscreen, avoiding direct sun exposure and wearing a hat when outdoors can prevent photosensitivity side effects, Patel says.

To manage her symptoms, Hazelton applied organic shea butter daily and wore gloves and socks to bed to keep her skin as moisturized as possible. “My nails were actually amazing during treatment because I was moisturizing them so religiously,” she says. “Whatever your skin can drink up during that time that is nontoxic, contains no preservatives and has no scents will help.”

For patients who are undergoing targeted therapy or immunotherapy, dealing with side effects can be more complicated. “In general, the newer cancer therapies (like immunotherapy) have very specific side effects, which may require specific treatment,” Patel says. “Patients have to go into it with the mindset that they will have some sort of side effect and ask their treatment team what to expect and what they can do to minimize it.”

LeBoeuf recommends that patients seek the advice of a dermatologist, who can work with the medical oncologist to manage skin side effects. “Whenever possible, if a specific dermatologic diagnosis can (be) made, then the most appropriate treatment can be instituted as quickly as possible,” LeBoeuf says. “If you treat the side effect specifically, based on dermatologic literature, then often, patients will recover and can continue their cancer treatment, as well.”

Both Patel and LeBoeuf urge that the mental and emotional side effects of treatment-related skin conditions receive as much attention as the physical side effects. “Often, patients are embarrassed or they feel like they shouldn’t be concerned about a side effect that isn’t life-threatening,” LeBoeuf says. “But the reality is, if a side effect affects your quality of life, increases your stress or changes your course of treatment, it can also affect your ability to fight the cancer. So be open, ask for help and advocate for referrals if you feel you aren’t getting the treatment you need.” Maintaining skin health can also prevent secondary problems such as infections or ulcers and can minimize scarring.

Taking one day at a time helped Hazelton maintain a positive mindset. “Thinking ‘Am I going to burn during radiation?’ or ‘What’s my scar going to look like?’ doesn’t help you mentally. Just approach it as it comes,” she says.

Patients might be surprised to learn that there is a silver lining to skin-related side effects: “Skin side effects to some of the newer targeted and immune therapies is correlated to having a better tumor response,” Patel says. “It is correlated to the body’s immune system being revved up to fight the cancer.”

Post treatment, patients should be advised about options to help restore the health of their skin, hair and nails. “What happens after the treatment isn’t talked about as much,” Dr. Anthony Rossi, a dermatologist and surgeon specializing in skin cancer, lasers and cosmetic surgery at Memorial Sloan Kettering Cancer Center, says. “Radiation can cause chronic changes in the skin; surgery, obviously, leaves a lot of scars, and high-dose steroids can leave stretch marks that last forever.”

To combat these side effects, Rossi and his team at Memorial Sloan Kettering are working on what they call “restorative oncodermatology,” which, he says, aims “to restore patients who go through cancer treatment and help them get back the form and function of their skin.”

Through the use of treatments such as topical retinoids, topical skincare, chemical peels, lasers, botulinum neurotoxin (such as Botox) and platelet- rich plasma (or PRP) injections — an experimental therapy that uses injections of the patient’s own blood platelets to prompt hair growth — Rossi reports that many survivors are seeing life-changing results. “There is definitely a cosmetic aspect that people want to improve... but these treatments help with skin functionality and overall quality of life, most importantly,” he says.

Hazelton has received chemical peels and laser treatments to reduce her wrinkles and melasma, as well as used over- the-counter eyelash serums to help her hair grow back.

“I thought my life was over when I got my diagnosis. I thought I would never look the same or feel the same ... but there are people out there to help you fix these physical things that remind you of your sickness,” she says. “A lot of people bounce back more than they expect they will. ... Your body really does recover; you just have to give it a chance.”

Although many cancers are unseen, different types of treatment can lead to painful, visible side effects, particularly when it comes to an individual's skin. 

Coping With Cancer Treatment’s Effects on Skin

When Jane Perlmutter learned in 1985 that she had mucinous carcinoma in her right breast, what struck her most was the veil of secrecy surrounding the disease.

“I was diagnosed in my early 30s, and at that time, cancer was still the C-word that people whispered about,” Perlmutter says. “And the word ‘breast’ was rarely even published.”

While Perlmutter received treatment at Memorial Sloan Kettering Cancer Center (MSK) in New York City, she recalls, a volunteer from the American Cancer Society’s peer-to-peer support program, Reach to Recovery, came into her room. “She told me that she was a 17-year survivor, and that was so powerful to me,” Perlmutter says. “That was the first time I really believed that I could survive this, so after I finished my treatment, I felt as though I was chosen to get involved.”

In the 35 years since, Perlmutter has been heavily involved in cancer advocacy and said she owes her activity, in part, to those who laid the groundwork. The breast cancer patient advocacy movement officially got its start in the early 1950s, and in the past 70 years, the disease has morphed from a shameful secret to a prominent issue. Awareness has grown about the importance of early detection and treatment and the need for dedicated research and care programs. And breast is the cancer that garners the most funding through U.S. nonprofit organizations, reaping $460 million in donations in 2015 alone.

Over the decades, the money brought in through rising awareness has helped researchers identify a variety of types of breast cancer; move from a standard treatment of radical mastectomy to the possibility of lumpectomy; and add chemotherapy, hormonal medications, targeted drugs and immunotherapy to the list of available options.

At its core, the breast cancer patient advocacy movement can be credited to a handful of brave women who first came forward with their stories, founding support groups and, eventually, political activism groups. Their goal: Raise awareness and money, and bring the patient experience into the conversation among scientists and clinicians to affect cancer culture, drug development, treatment options, Food and Drug Administration (FDA) regulations and more.

Terese Lasser was one of the first women to kick-start the movement when, in 1952, she went to MSK to have a malignant breast lump biopsied under general anesthesia. She awoke to find herself, in her words, “bound like a mummy in surgical gauze,” and learned that a radical mastectomy had been performed without her knowledge or verbal consent.

Emotionally devastated by the mastectomy, Lasser also became infuriated with the cavalier attitude of her male surgeon, who shrugged off her questions about prosthesis options and rehabilitation exercises. Turning her anguish into action, Lasser founded Reach to Recovery in 1953 to bring women with breast cancer together to address the needs that their surgeons did not view as important. In 1969, the program that would inspire Perlmutter to join the fight was incorporated into the American Cancer Society.

In the early days of breast cancer advocacy, the biggest obstacle was the lack of open discussion. Patients like Lasser cleared that hurdle, and public figures took the cause a step further. Child movie star Shirley Temple Black became the first woman to openly write about her experience with breast cancer in 1972 in a monthly women’s magazine, famously stating: “The doctor can make the incision; I’ll make the decision.”

In 1974, first lady Betty Ford shared news of her breast cancer diagnosis and radical mastectomy with the American public through a televised press conference; just three weeks later, Happy Rockefeller, the wife of Vice President Nelson Rockefeller, disclosed the discovery of a malignant lump in her own breast.

Patient and advocate Rose Kushner boosted the movement in 1975 by publishing “Breast Cancer: A Personal History and an Investigative Report.” In her book, Kushner took issue with radical mastectomies and with performing biopsy and mastectomy together instead of delaying treatment until a patient had time to consider her options.

That public discussion led to women learning more about self-examination and medical options, but real change in the doctor’s office came with the rise of female surgeons, including Dr. Susan Love and Dr. Janet Osuch.

“When I went into surgery, and even when I went to medical school, there were quotas for how many women they would take,” Love says. “When I finished, nobody would offer me a job because nobody wanted a woman, so I started my own practice. And the only patients they would send me were women with breast problems.”

In 1985, Dr. Bernie Fisher, a surgeon and pioneer in the breast cancer patient advocacy movement, published an article comparing total mastectomies with lumpectomies, a form of breast preservation. Through a randomized study of stage 1 and 2 breast tumors, Fisher found that lumpectomy was at least as effective as mastectomy in treating breast cancer in these patients. Although his findings were instrumental in giving women a choice, it was primarily female surgeons like Love and Osuch, whose practices were soon flooded with patients who had breast cancer looking for options, who made change possible.

“I started doing breast preservation as soon as Fisher published that article, and that was not what most male surgeons were doing,” Osuch says. “But that was part of what attracted women to my practice. They came from all over Michigan. Women brought something really special to the field of surgery because they tended to listen to their patients more.”

In 1986, the state of Michigan amended its Public Health Code to state that “a physician who is 

administering the primary treatment for breast cancer ... shall inform the patient, orally
and in writing, about alternative methods of treatment,” serving as just one example of how advocacy turned into action.

Good timing also helped drive breast cancer patient advocacy. In the early 1980s, two move- ments seemed to collide, setting the stage for women to organize around breast cancer like never before.

“When women were growing up, you were a good girl if you didn’t say anything if you didn’t challenge authority,” Osuch says. “You basically went along to get along — until the early ’80s, with the rise of the feminist movement and as women watched the
AIDS activists.”

The AIDS movement was one of the first disease-specific efforts to increase research and funding, sometimes using aggressive and “in-your-face” tactics, and women took note. In 1980, Nancy Brinker was watching as her sister Suzy died from breast cancer at the age of 36. “I can’t tell you what that did to me,” Brinker says. “It changed my world, my entire life.”

At the end of her life, Brinker’s sister made her promise to do everything in her power to end breast cancer, which led Brinker to launch the Susan G. Komen Breast Cancer Foundation — now simply known as Susan G. Komen, named after her sister — in 1982.

The creation of the organization, just two years before Brinker’s own breast cancer diagnosis, marked the first public effort to raise money specifically for breast cancer, tearing a page out of the AIDS playbook. Susan G. Komen’s Race for the Cure, which began in 1983, quickly became one of the world’s largest 5Ks dedicated to breast cancer awareness. Susan G. Komen itself has become the largest and most-funded nongovernmental breast cancer organization in the United States.

Besides playing a role in equating the color pink with breast cancer awareness and support, Susan G. Komen has raised and given away about $3 billion for research, as well as launched a community approach to detection 

and care. In addition to allocating $1 billion to fund
the careers of young scientists, many of whom are now the leaders of some of the best labs in the country, the organization funneled the other $2 billion directly into over 104 communities around the world, where Susan G. Komen teaches women about the disease, funds, detection, care and support programs, and helps people with any stage of breast cancer find and participate in clinical trials.

The AIDS movement not only revealed the effectiveness of disease-specific efforts, but it also helped women realize they could have educated and informed conversations with scientists and surgeons, with the ultimate goal of influencing breast cancer policymaking.

In 1991, several advocacy groups formed the National Breast Cancer Coalition (NBCC), “a collaboration of activists, survivors, researchers, policymakers, grassroots groups and national organizations that have come together as disruptive innovators for social change.” The group’s mission is to link organizations across the country to give them a voice in government, laboratory and community decisions, and initiatives related to breast cancer.


“The NBCC was really instrumental in getting research funding, and they were the main movers in saying: ‘We played a key role in getting this funding, and we want a seat at the table to have an influence in how the research is done,’ ” Perlmutter says. “NBCC trained advocates so they understood enough of the science to be good contributors, and then researchers began to realize that we were smart, good people who had a different perspective to share, and they began to engage us in peer review and research.”

The involvement of advocates in research, she says, was a groundbreaking advance; for example, clinical trials designed purely from a scientist’s perspective can fail due to conditions or requirements that aren’t comfortable for patients, leading to low enrollment or nonadherence.

“Getting advocates involved early to tell scientists how patients are likely to react to a trial design will make a difference in how well the trial does,” Perlmutter says.

Efforts like these resulted in greater understanding of the value of patient engagement and encouraged its widespread use. Organizations that now operate with patients top-of-mind include the Patient-Centered Outcomes Research Institute (PCORI), which funds studies that
offer patients and caregivers the information they need to make important health care decisions, and the FDA’s Patient-Focused Drug Development program, defined on the FDA website as a “systematic approach to help ensure that patients’ experiences, perspectives, needs and priorities are captured and meaningfully incorporated into drug development and evaluation.”

Over the course of 70 years, the coordinated breast cancer patient advocacy movement pulled the disease out of the shadows and into the spotlight, turning breast cancer into the most funded cancer in the country. So, what’s next?

“Breast cancer advocacy took breast cancer out of the closet, and it made people recognize that many cancer patients are basically cured when they are detected early,” Perlmutter says. “But metastatic breast cancer survivors feel as though they have not been represented. Most of the funding goes to early-stage breast cancer, but if you can prevent metastasis, you can make a bigger impact.”

When she learned in 2005 that she had stage 2a ductal carcinoma in situ, Dian “CJ” Corneliussen was initially impressed with the support she received. However, when her cancer metastasized to her lung a year later, her experience was radically different.

“When I was diagnosed with primary breast cancer, I was inundated with offers of support and friendship,” Corneliussen says. “When I metastasized a year later, I was told by my oncologist to expect death in two to three years and then told by the breast center staff — (the place where) I was diagnosed and frequently sought emotional support — to avoid the center and hide my diagnosis from other patients. It was all too depressing and would frighten 

other patients to learn that actually happened. There was no support, no one to talk to, and then I learned there was virtually no research about metastatic breast cancer.”

Corneliussen felt that the breast cancer movement pushed the concept that patients only experienced metastasis if they “did something wrong,” she says. Feeling isolated and alone, as well as shocked at the contrast between her two experiences, she decided to found METAvivor, a metastatic breast cancer research, support and awareness organization, in 2009.

According to METAvivor, although 30% of people who receive a diagnosis of early stage breast cancer will eventually experience metastasis, a minority of funds raised for breast cancer research are directed at disease that has spread.

“We’re trying to expand the culture of breast cancer,” Corneliussen says. “It isn’t just about your breast and whether you get to keep it or lose it. It’s about your life.”

The movement also has begun focusing more on young survivors and their issues related to fertility and sexuality that are unique to their age. And, there is also a growing understanding and awareness of survivorship or living with an inherited predisposition to breast cancer. Previvors who test positive for breast cancer-associated gene mutations are closely observed through regular screening so that any cancers will be detected early; they are also asked to consider preventive surgeries, such as the removal of their breasts, ovaries and fallopian tubes. 

For more than 15 years, patients and previvors with these gene mutations have been able to turn to Facing Our Risk of Cancer Empowered (FORCE), the only national nonprofit organization dedicated to supporting them. The group includes resources for men, who can be affected by or pass down these inherited genes.

Finally, the issue of health equity and disparity in care is a major focus across the entire breast cancer patient advocacy network. More sisters networks focused specifically on Black and Hispanic patients with breast cancer have formed, and there is a push toward ensuring that advocates are compensated for their time, which would help make advocacy involvement, which has traditionally been volunteer based, more representative of the entire population.

For Brinker, addressing health equity is the next necessary step toward fulfilling the promise she made to her sister 40 years ago — and what led her recently to found the Promise Fund of Florida, with the goal of eliminating barriers to quality health care and creating a community- based continuum of care for all.

“I have committed the rest of whatever my life will be to get this done,” Brinker says. “At the end of the day, I honestly believe that sometimes, to be an advocate, you have to be a bit of a maverick and just go off and do what you think is the right thing to do.”

The breast cancer patient advocacy movement that started in the 1950s pulled the disease out of the shadows, bringing patients’ options to light.

From Secrecy to Support: How the Breast Cancer Patient Advocacy Movement Pulled the Disease Out of the Shadows

Four years ago, Ronda Beaty was in the best shape of her life, so when she found herself winded just from walking up the stairs, she knew something was wrong. “My boyfriend and I were hiking in an Ohio state park one day, and I couldn’t even keep up with him,” says Beaty, who lives in Tipp City, Ohio. “And I was used to being the one in front.”

After two pneumonia diagnoses, Beaty was ordered to get a CT scan — and that’s when her life changed. On Dec. 23, 2016, she was told she had adenocarcinoma non- small cell lung cancer (NSCLC). “It was stage 4 with metastases to my brain, adrenal glands and lymph nodes,” she says.

Beaty began treatment in January 2017, and although she credits her medical team for saving her life, she struggled with anxiety and feeling helpless throughout her treatment.

“I have a huge group of friends and family and loved ones that are a support network, but none of them are lung cancer patients,” Beaty says. “I just felt like I really needed to talk to somebody that understood — (who) didn’t think they understood but really understood.”

But Beaty couldn’t find anyone like that. Time passed, and in December 2019 — almost three years to the date of her diagnosis &mdash; she felt ready to give up. Then she came across the website for GO2 Foundation for Lung Cancer, the world’s leading organization dedicated to extending and improving the lives of individuals who have or are at risk of lung cancer.

“I emailed them, and the very next morning, I had an email in my inbox — a personal introduction about who they were, what they were about and how they could help me,” she says.

Beaty was quickly connected with a woman through the organization’s Phone Buddy Program, which offers peer- to-peer support and matches patients with volunteers who have either been through or are going through lung cancer treatment. Beaty was surprised to learn that her mentor, Sandy Spears, was not only a stage 4 lung cancer survivor but also had no evidence of disease (NED). “I didn’t even know it was possible to be a stage 4 lung cancer patient with NED,” Beaty says.

The two exchanged texts for weeks, and then Spears asked if Beaty wanted to talk on the phone. “We spoke for about an hour-and-a-half on that first call,” Beaty says. “When I met Sandy, I had been battling for three years, and I was pretty ready to throw in the towel. But meeting her changed my whole attitude. I cried for three solid days because just meeting someone like that had given me newfound hope.”

Patients with lung cancer can get emotional, educational and social support through individual and group therapy sessions, online groups, and help hotlines, but options may be limited. “Even though lung cancer causes more deaths than breast, prostate and colorectal cancers combined,

the number of support groups formed for lung cancer survivors is surprisingly very small,” according to a study published in 

“There is still stigma out there, multiple types of stigma, that keeps people from getting support,” says Katie Brown, vice president of support and survivorship programs at LUNGevity, a national lung cancer-focused nonprofit organization. “That might be a deterrent for someone reaching out (for help), so maybe a peer mentor might appeal to them instead.”

Peer-to-peer support offers a unique benefit that other forms of support cannot: the ability to pair a patient with a mentor who has or has had lung cancer and is uniquely qualified to help them cope.

“The benefit is that you are specifically connecting with a peer mentor to talk about your disease,” Brown says. “A mentor is able to walk with you through it, encourage you and offer you hope, because they’ve been through it and came out on the other side.”

In addition to providing emotional support and sharing coping techniques, peer mentors can help shed light on treatment options, discuss how to deal with difficult conversations with doctors or family members, and help patients begin to visualize their life post-treatment.

LUNGevity’s LifeLine Support Partners program matches patients to mentors from a database of over 400 volunteers. Pairing is based on gender, age and diagnosis whenever possible. GO2’s Phone Buddy Program matches by stage, treatment plan, symptoms, side effects and profile, ensuring that patients and mentors are as compatible as possible.

“I was a little leery going into all of this,” Beaty says, “but it’s not intimidating at all. Sandy and I are the same age. She was diagnosed approximately at the same time I was. She had the same metastases that I have. We just had so much in common right away.”

Another benefit of customization: “It’s as personal as I want it to be,” Beaty says. “If I don’t feel up to it to respond, there’s no judgment there. You decide the level of commitment.” Although some patients prefer to converse only through text or email, others get close with their mentors, even meeting face-to-face.

Finally, mentors can open doors in ways that patients might never have imagined. In addition to helping Beaty find hope in her diagnosis, Spears introduced her to support programs, such as Lung Cancer Living Room, a GO2 Foundation support group that Beaty attends virtually through YouTube Live each month. Beaty’s involvement with GO2 Foundation and LUNGevity also led to a grant to attend LUNGevity’s annual International Lung Cancer Survivorship Conference, the world’s largest meeting of patients with lung cancer and caregivers, where she will be surrounded by even more patients, doctors and scientists.

“All of these people that I’ve met ... have instilled in me a new drive — a new reason, a new purpose to live,” Beaty says.

To become a mentor for GO2 Foundation and LUNGevity, volunteers simply need to have or have had lung cancer and be willing to speak with others about their experience. LUNGevity provides hopeful volunteers with online training videos, a manual and conference call training to ensure they are ready for the responsibility ahead.

Mentors choose to volunteer for a variety of reasons. Some want to pay it forward after receiving support of their own; others, like Spears, volunteer for the opposite reason — because they didn’t have support themselves.

“I decided to be a mentor in the Phone Buddy Program because when I was diagnosed with cancer, I didn’t have anybody to reach out to,” says Spears, who lives in Monroe, Georgia. “I felt totally alone.”

Lisa Zarov of Highland Park, Illinois, joined LUNGevity’s LifeLine Support Partners after surviving stage 1a adenocarcinoma NSCLC. A licensed clinical and school social worker, she felt uniquely qualified to help but also wished to stay connected to the cancer community.

“I just wanted to be able to do something, anything,” Zarov says. “I suppose it would have been easy enough to just have surgery, go on with my life and not look back, but I felt like in my position, as lucky as I was to have it caught early, there was no way I could just turn around and not look back.”

Nichelle Stigger, one of the patients Zarov came to support, received a diagnosis of stage 2b primary mucinous adenocarcinoma, one of the rarest types of lung cancer, at age 34. Stigger credits her relationship with Zarov as a major factor in her ability to work through her anger and shock at her diagnosis.

“It was really, really special,” says Stigger, who lives in Oak Park, Illinois. “I always tell Lisa, ‘I love you ... and know that I am better because you were able to love me through this process.’”

Anyone considering mentorship should consider a few factors, starting with their role in the relationship. “You are there to support the other person, not the other way around,” Zarov says. “Sometimes there’s a fine line between sharing your story and kind of crossing that line of leaning on the person you’re trying to support.”

Peer-to-peer connections bring unique benefits for patients coping with lung cancer.
 

Stacy Willingham

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