50 THE QUEENS COURIER • WELLNESS • FEBRUARY 21, 2019 FOR BREAKING NEWS VISIT WWW.QNS.COM
wellness
The Rarest of the Rare
What to know about a cancer you may not have heard of
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.
Rare Disease Day is recognized on the
last day of February each year and serves
as an important time to bring awareness
to soft -tissue sarcomas such as 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 eff ective,
tumor-specifi c 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. Th ere
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 aff ect 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 defi ned 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 Th ey Impact Diagnosis?
ES can take two forms:
Distal-type: Classic form, typically
aff ects 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
aff ects 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 identifi cation
and diagnosis more diffi cult.
Dealing with a Diagnosis?
Due to its rarity, harmless appearance
in its initial stages and occurrence in
young people, ES is oft en misdiagnosed,
according to the Liddy Shriver Sarcoma
Initiative. For this reason, the Sarcoma
Foundation of America recommends
checking oft en 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. Th ese 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. Th e specialist
may confi rm 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
aft er 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 fi eld 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.
An ES Diagnosis Journey
In the spring of 2008, Maria Voermans’
4-year-old daughter requested an “airplane
ride,” and as Voermans lift ed the
young girl up with her legs, she had to
make an “emergency landing” because of
some sudden and signifi cant pain in her
upper right thigh.
Aft er 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.
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
Courtesy Family Features
Photo courtesy of Getty Images
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