38 THE QUEENS COURIER • HEALTH • JANUARY 2, 2020 FOR BREAKING NEWS VISIT WWW.QNS.COM
What you need to know about rare cancers
Although rare cancers don’t occur oft en,
they can aff ect people of all ages and genders.
A rare cancer is defi ned as fewer than
15 new diagnoses per 100,000 people per
year, according to the National Cancer
Institute (NCI). Additionally, as noted
by the American Cancer Society (ACS),
the 5-year survival rate is lower for people
diagnosed with a rare cancer than for
people living with more common cancers.
Greater awareness of rare cancers may
lead to earlier diagnosis and management,
and potentially better survival rates.
1. Th ere are more than 50 types of soft
tissue sarcomas (STS). STS account for
about 1 percent of all cancers and aff ect
soft tissues such as muscle, fat, nerves,
blood vessels and skin. Although STS can
be found in any part of the body, they are
oft en in the arms or legs, internal organs,
the back of the abdominal cavity or in the
trunk, head and neck area.
If you have recently been diagnosed
with STS, it’s important to ask your doctor
for more information about the specific
sub-type you have. For example, if you
received a diagnosis of undiff erentiated
sarcoma, ask your doctor for an integrase
interactor-1 (INI1) test to see if you have
a rare STS called epithelioid sarcoma (ES).
(See sidebar for more on ES.)
2. STS can be visible or invisible depending
on location. STS may appear as painless
bumps under the skin, usually on
arms or legs. Some sarcomas begin in the
abdomen and typically don’t show symptoms
until they grow and press on nearby
organs, nerves, muscles or blood vessels.
When this occurs, symptoms may include
pain and trouble breathing.
3. Early diagnosis can help inform disease
management. As with other types
of cancer, early diagnosis of STS is key,
as earlier treatment may result in more
favorable outcomes. Because other conditions
can cause similar symptoms, it’s
important to check with your doctor if
you are experiencing any of the warning
signs listed above. If your doctor decides
it’s best to “watch and wait,” consider
developing a six-week follow-up plan in
partnership with your health care team if
your symptoms have not improved.
4. Seeking care from a specialist is key.
Given the rarity of STS, fi nding a sarcoma
specialist who understands the complexity
of this rare disease and can help
determine which treatment option is best
for you is important. Treatment options
depend on multiple factors, including
your overall health, the location and type
of tumor, its size and whether the disease
has spread elsewhere in the body. STS are
typically treated with a combination of
options including chemotherapy, radiation
therapy and surgery. A specialist may
also speak to you about participating in a
clinical trial where investigational therapies
in STS are being studied.
Learning more about epithelioid
A rare type of STS, epithelioid sarcoma
(ES) accounts for less than 1 percent
of all STS, which themselves account
for approximately 1 percent of all cancers,
according to research published
in “Archives of Pathology & Laboratory
Medicine.” ES can present as a lump or
sore on the skin.
Notably, more than 90 percent of
ES tumors do not express the INI1
protein, which when present acts to
suppress tumor growth. INI1 loss
plays an important role in the diagnosis
of ES, according to researchers
with “Th e American Journal of Surgical
Data from the NCI indicates that
approximately 150-200 people in the
United States are diagnosed with ES
each year. Research published in “Th e
Journal of Clinical and Aesthetic
Dermatology” found the disease oft en
occurs in young adults in their 20s and
30s. Because most ES patients are adolescents
and young adults, there is a
gap in the unique psycho-social needs
for this patient population, including
resources for patients who miss school
while undergoing treatments, as well as
fertility considerations later in life.
If you or someone you love is living
with ES, you can fi nd resources, information
and the real-life perspective of
an ES survivor at ESsentialsforES.com.
— Courtesy of Family Features