The Rarest of the Rare

What to know about a cancer you may not have heard of

(Family Features) A woman celebrating her 40th birthday, a young boy starting second grade or a college grad about to begin his career. All three could develop a rare form of cancer known as epithelioid sarcoma (ES), a form of soft-tissue sarcoma.

There is little information available about ES. Patients, advocates, doctors and researchers across the United States are aiming to educate people about this ultra-rare cancer and the unmet need for an effective, tumor-specific treatment. Consider these facts about ES:

What are Soft-Tissue Sarcomas and What is Epithelioid Sarcoma?
Soft-tissue sarcomas (STS) are a type of cancer that occur in tissue like muscle, fat, skin, nerves and blood vessels. There are more than 50 types of STS, including ES, which is a rare form that can occur under the skin in the limbs (such as the arms, hands, legs or feet) or soft tissue in other places like the abdomen. Adults in their 20s and 30s and men, in particular, are more likely to get ES, although it can affect people of all ages and genders, according to research published in the Journal of Clinical and Aesthetic Dermatology.

How Rare is Rare?

According to the American Cancer Society, a rare cancer is defined as fewer than six new diagnoses per 100,000 people per year.

ES is an ultra-rare cancer. According to available epidemiology and case reports, it is estimated about 600 people are properly diagnosed in the U.S. and Europe each year.

What are the Most Common Types of ES and How Do They Impact Diagnosis?
ES can take two forms:
Distal-type: Classic form, typically affects teenagers and young adults.

• Typically forms under the skin in areas like hands, legs and feet.
• Can be mistaken for another skin condition like an infected wart or a wound that won’t heal, which may delay diagnosis.
• Proximal-type: Rarer form, mainly affects adults and is more aggressive.
• Typically forms in central areas of the body like the abdomen.
• Can be mistaken for menstrual cramps or an upset stomach, making identification and diagnosis more difficult.

Dealing with a Diagnosis?
Due to its rarity, harmless appearance in its initial stages and occurrence in young people, ES is often misdiagnosed, according to the Liddy Shriver Sarcoma Initiative. For this reason, the Sarcoma Foundation of America recommends checking often for lumps and bumps in places they shouldn’t be.

For people faced with a sarcoma diagnosis, it’s important to get a second opinion from a sarcoma specialist. These specialists have extensive knowledge of STS and can determine what form of sarcoma one may have, what stage it is and the best course of treatment. The specialist may confirm the diagnosis with a physical examination, a scan or a tissue sample (biopsy) of the area.

It’s common to feel a range of emotions after a diagnosis of ES, according to Clear View Health Partners, including:

• Fear
• Denial
• Frustration
• Anxiety
• Hope that treatment will be successful
• Determination to beat ES

What Treatment Options are Available?
Treatment options depend on a variety of factors, including the location of the tumor, tumor staging and whether the disease has metastasized or spread to other parts of the body.

For patients with early stage ES, many elect to have surgery to remove the tumor, which may precede or be followed by radiation therapy or chemotherapy treatment, according to the Journal of Clinical and Aesthetic Dermatology. If the cancer returns or spreads, a patient may undergo radiation therapy and chemotherapy. New treatment options are being studied through clinical research, which is why seeking a specialist in the field is important if one is faced with a diagnosis.

As with many cancers, early detection is important and can increase survival or successful treatment. Typically, the distal form of ES is associated with more favorable survival rates than the proximal form.

4 Things to Do to Address ES Today

1. Don’t ignore your bumps and lumps, see a doctor as soon as possible.

• curesarcoma.org/patient-resources/sarcoma-infographic/  

2. Learn more about epithelioid sarcoma and its symptoms.

• mayoclinic.org/diseases-conditions/epithelioid-sarcoma/cdc-20392420

3. Seek a second opinion.

• sarctrials.org/sarc-centers-map

4. Find support if you’re faced with a diagnosis.

• sarcomaalliance.org/resources/support-groups
• cancersupportcommunity.org
• cancercare.org/counseling

An ES Diagnosis Journey

In the spring of 2008, Maria Voermans’ 4-year-old daughter requested an “airplane ride,” and as Voermans lifted the young girl up with her legs, she had to make an “emergency landing” because of some sudden and significant pain in her upper right thigh.

After a few months, the pain persisted. Voermans continued to jog and play sand volleyball, thinking nothing of it. At the recommendation of her primary care physician, she took some anti-inflammatories and tried to rest, which wasn’t easy to do as a single mother of two young children.

Two more months went by and her leg caused increasing problems. She could feel something in her leg, but never considered it a “lump” because it was not visible on the outside. Voermans took matters into her own hands and visited a sports medicine orthopedic specialist for further testing.

An MRI found a mass in her right leg and she was referred to one of the few musculoskeletal oncologists in Wisconsin, her home state. He ordered a biopsy, which on Voermans’ youngest daughter’s third birthday confirmed her worst fear: it was a rare form of cancer called proximal-type epithelioid sarcoma, and it was stage three. Her biggest concern was not living to experience future holidays, birthdays, graduations and other life milestones with her daughters.

Voermans underwent chemotherapy, radiation therapy and had surgery to remove the tumor. As of July 2018, Voermans reports the cancer has not returned.

Today, she’s a wellness coordinator supporting other people diagnosed with cancer who are undergoing treatment or post-treatment. She’s able to use her own cancer journey to provide empathy to others, and it’s brought satisfaction to the whole experience.

Content courtesy of Epizyme, Inc.

Photo courtesy of Getty Images (Doctor talking to man)

There is No One-Size-Fits-All Breast Cancer

(Family Features) Breast cancer. It is a simple phrase representing a complex journey thousands of women travel each year. Even during October, when Breast Cancer Awareness Month brings attention to the disease, many people don’t realize breast cancer is not just one disease; it’s extremely complex and classified into different types. Understanding the many types of breast cancer is important – not just for the 1-in-8 women who will be diagnosed in their lifetime, – but for those who love them, too.¹

Laura Ross knows first-hand. She was diagnosed with triple-negative, stage I breast cancer when she was 41 years old.

“No one in my family had breast cancer and I had no information,” Ross said. “I had not even heard breast cancer had different types until the results came back from my biopsy.”

Unprepared and in shock, she relied heavily on her support circle, which in addition to her doctor and healthcare team, helped her learn more about her diagnosis. Empowered by this information, Ross had the confidence to make informed treatment decisions with her doctor.

You or someone you love may be affected by breast cancer in your lifetime and have to help make a treatment decision. To change the perception that breast cancer is just one disease, Living Beyond Breast Cancer, Genentech and ThirdLove created the “Not One Type” campaign, which offers these tips:

1. Understand the basics. Breast cancer is classified into different types based on the unique characteristics of each tumor (size, lymph node status, stage and subtype, among others). The tumor’s size, the status of whether the cancer is in the lymph nodes and the cancer’s stage, which indicates whether or not it has spread, are important factors in helping determine a diagnosis. The subtype, which is determined by studying the cancer cells using diagnostic tests, more specifically defines the type. Some common breast cancer subtypes include hormone receptor-positive, HER2-positive and triple-negative. These are example subtypes. A patient could also have a combination of subtypes, and/or other factors contributing to cancer growth.

2. Ask your doctor specific questions. If you or a loved one is faced with a diagnosis, knowing some questions to ask a doctor makes all the difference. Consider these questions to start:
   • How large is the tumor?
   • Has the cancer spread to the lymph nodes?
   • What is the stage?
   • What is the HER2 status? Hormone receptor status?
   • Why do you recommend this particular treatment plan? Are there other treatment options to consider?
   • Can you explain what these medicines do to the cancer in the body?
   • What are the common side effects of treatment?

3. Pass it on. Share this information with a loved one or friend. Understanding that breast cancer is not a one-size-fits-all-disease can help those directly affected by breast cancer and those who may be impacted in the future make more informed decisions.

During October – and year-round – arm yourself with knowledge about the different types of the disease and use that information to be a more empowered patient or caregiver. Visit NotOneType.org to learn more.

Photo courtesy of Getty Images

¹ American Cancer Society. Cancer Facts & Figures 2018. Atlanta: American Cancer Society; 2018.

² Kohler BA, et al. Annual Report to the Nation on the Status of Cancer, 1975–2011, Featuring Incidence of Breast Cancer Subtypes by Race/Ethnicity, Poverty, and State. J Natl Cancer Inst 2015;107(6):djv048.

10 Steps to Help Prevent Cancer

(Family Features) Nearly 4 out of 10 Americans will be diagnosed with cancer in their lifetimes, and it remains the second-leading cause of death for Americans, but nearly half of all cancer cases can be prevented.

Research from the American Institute for Cancer Research (AICR) shows that diet, exercise and weight play a critical role in cancer prevention.

“Making changes in what you eat, being physically active and maintaining a healthy weight have strong and clear links to your risk for cancer,” said Alice Bender, MS, RDN and director of nutrition programs at AICR. “We know from decades of research and a thorough review of the science that there are simple things we can all do to reduce our risk.”

To live a cancer-preventive lifestyle, consider taking these 10 steps recommended by the scientific experts at AICR:

1. Be a healthy weight. Higher body fat can be a cause of many cancers. Try to stay at a healthy weight and avoid weight gain as you get older.
2. Be physically active. Incorporate moderate physical activity into your daily life through steps like walking more and sitting less.
3. Eat a diet rich in whole grains, vegetables, fruits and beans. Make these foods a major part of your diet.
4. Limit consumption of “fast foods” and other processed foods high in fat, starches or sugars. Cut down on processed foods to help control calorie intake and maintain a healthy weight.
5. Limit consumption of red and processed meat. Eat no more than three portions of red meat per week, and little – if any – processed meat.
6. Limit consumption of sugar-sweetened drinks. Don’t drink sugar-sweetened drinks, which contribute to weight gain. Choose water instead, when possible.
7. Limit alcohol consumption. For preventing cancer, it’s best not to drink alcohol.
8. Do not use supplements for cancer prevention. Aim to meet nutritional needs through diet alone.
9. For mothers, breastfeed your baby, if you can. Breastfeeding is good for both mother and baby.
10. After a cancer diagnosis, follow these recommendations, if you can. Cancer survivors are encouraged to continue following these guidelines.

Refraining from smoking, avoiding other exposure to tobacco and limiting sun exposure are also important in reducing cancer risk.

Because it can be hard to make lifestyle changes, AICR aims to help people adopt healthier behaviors through efforts like the Cancer Health Check, a tool that shows people how their lifestyle stacks up against known cancer risks and recommends changes that can improve health.

For recipes, tips and other resources, visit aicr.org.

Photo courtesy of Getty Images

Treatment Options for Men with Enlarged Prostate

(Family Features) If you’re a male over the age of 45, chances are you may be suffering from a condition more common than prostate cancer – benign prostatic hyperplasia (BPH). More commonly known as enlarged prostate, BPH can cause bothersome urinary symptoms that can worsen with age. In fact, nearly 40 million men in the United States are affected by enlarged prostate, according to research published in the “Journal of Urology.”

During Men’s Health Month, consider these steps from the experts at NeoTract, Inc., manufacturer of the UroLift System, for alleviating enlarged prostate symptoms:

Signs You Should See a Urologist

An enlarged prostate obstructs the bladder opening and can lead to a myriad of bothersome urinary symptoms. Symptoms of BPH include frequent urination, a weak or slow urine stream, incomplete bladder emptying, difficulty or delay in starting urination and a urine stream that stops and starts. It’s important to see a physician if any of these problems arise or persist.

Enlarged prostate can also cause loss of productivity and sleep and, in some cases, can lead to depression. According to a survey sponsored by NeoTract, one of the most common symptoms of BPH – interrupted sleep – is also impacting men’s partners. Sixty-four percent of women surveyed who were affected by their partners' BPH symptoms said it impacts their sleep, too.

Traditional Treatment Options

Medication is often the first-line therapy for enlarged prostate, but relief can be inadequate and temporary. Some patients may suffer uncomfortable side effects from the medications, including dizziness, headaches and sexual dysfunction, which can prompt them to quit using the drugs.

“Medical and surgical treatments for BPH ranging from medications to surgery have been used for decades with varying degrees of success and side effects,” said Dr. David O. Sussman, DO, FACOS. “Medications can be helpful in relieving symptoms for some men, but patients must continue taking them long-term to maintain the effects.”

The classic alternative for patients who opt against medication is surgery that cuts, heats or removes prostate tissue to open the blocked urethra. Sussman said surgical options such as transurethral resection of the prostate or photovaporization of the prostate are usually effective. However, these options typically require general anesthesia, overnight hospitalization and post-operative catheterization. Surgery can also increase the risk of erectile dysfunction or loss of ejaculation.

An Alternative Treatment Method

Another option for men looking to relieve their BPH symptoms without undergoing major surgery or taking long-term BPH medications is the UroLift System treatment, a minimally invasive procedure that takes less than an hour and doesn’t require any cutting, heating or removal of prostate tissue.

A urologist uses the device to lift and move the enlarged prostate tissue out of the way so it no longer blocks the urethra (the passageway that urine flows through). Tiny implants are then used to hold the tissue in place, leaving an unobstructed pathway for urine to flow through normally.

Most common side effects are mild-to-moderate and include pain or burning with urination, blood in urine, pelvic pain, urgent need to urinate or the inability to control the urge. The procedure has a low catheter rate and most symptoms resolve within 2-4 weeks after the procedure.

To learn more, visit UroLift.com .

Photo courtesy of Getty Images (Man at the doctors)