Sarcomas are rare types of cancer that develop in
the supporting structures of the body, such as bone,
muscle or cartilage. There are two main types of
sarcoma: soft tissue sarcomas and bone sarcomas.
Soft-tissue sarcomas can develop in muscle, fat,
blood vessels or in any of the other tissues that
support, surround and protect the organs of the
body. Bone sarcomas can develop in any of the bones
of the skeleton but may also develop in the soft
tissue near bones.
Soft tissue sarcomas
Soft tissue sarcomas are rare tumours. Their annual
incidence is around 2-3/100,000, and they account for
less than 1% of all malignant tumours. In paediatric
age, however, about 8% of all tumours are soft tissue
Half of the paediatric soft tissue sarcomas are
rhabdomyosarcomas, half constitute the heterogeneous
group of the so called "non-rhabdomyosarcoma" soft
Rhabdomyosarcoma is the most common of the soft
tissue sarcomas with approximately 5-6 children per
million diagnosed each year. Most of them are
younger than 10 years old. It is more common in boys
These tumours develop from muscle or fibrous tissue
and can grow in any part of the body. The most
common areas of the body to be affected are around
the head and neck, the bladder or the testes.
Sometimes tumours are also to be found in a muscle
or a limb, in the chest or in the abdominal wall.
Occasionally, if the tumour is in the head or neck
region, it can spread into the brain or the fluid
around the spinal cord.
Causes of rhabdomyosarcoma
The cause of rhadomyosarcoma is unknown. Research is
going on all the time into possible causes of this
disease. Children with certain rare genetic
disorders, such as Li Fraumeni syndrome, have a
higher risk of developing rhabdomyosarcoma.
Signs and symptoms
The signs and symptoms will depend on the part of
the body that is affected by the rhabdomyosarcoma.
The most common sign is a swelling or lump. If the
tumour is in the head area it can sometimes cause
blockage (obstruction) and a discharge from the nose
or throat. Occasionally an eye may appear swollen
and protruding. If the tumour is in the abdomen (tummy)
the child may have discomfort in the abdomen and
problems going to the toilet. If the tumour is in
the bladder, the child may have blood in the urine
and have difficulty passing urine.
How it is diagnosed?
A variety of tests and investigations may be needed
to diagnose a rhabdomyosarcoma. These usually
include a biopsy. This involves a small operation to
remove a sample from the tumour to be looked at
under a microscope. A biopsy is usually done under a
general anaesthetic. Various tests may be done to
check the exact size of the tumour and whether it
has spread to any other part of the body. These may
a chest x-ray to check the lungs
CT or MRI scans
blood and bone-marrow tests
The 'stage' of a cancer is a term used to describe
its size and whether it has spread beyond the part
of the body from which it originated. Knowing the
particular type and the stage of the cancer helps
the doctors to decide on the most appropriate
Most patients are grouped depending on whether the
cancer is found in only one part of the body (localised
disease) or whether the cancer has spread from one
part of the body to another for example the lungs or
bones (metastatic disease). The place in the body
where the rhabdomyosarcoma started is also important
information that is taken into account in the
Treatment depends upon some patient and tumour
characteristics such as the size of the tumour, the
subtype (when examined under the microscope) its
position within the body, the age of the child or
young person and whether it has spread. The
treatment of rhabdomyosarcoma usually includes
surgery, radiotherapy or chemotherapy, or a
combination of these.
If possible, surgery will be used to remove the
tumour, however if it cannot be easily removed then
it is often better to shrink it down with
chemotherapy before attempting further surgery.
Chemotherapy using a combination of drugs is often
given before surgery to shrink the tumour.
Chemotherapy is the use of anticancer (cytotoxic)
drugs to destroy cancer cells. Your doctor will give you more
details about the chemotherapy that will be used.
Radiotherapy may be given to the area of the tumour.
If the tumour cannot be removed with surgery the
treatment will usually involve a combination of
chemotherapy and radiotherapy.
Radiotherapy treats cancer by using high-energy rays
that destroy the cancer cells, while doing as little
harm as possible to normal cells.
Side effects of treatment
Treatment for rhabdomyosarcoma often causes side
effects, and your child's doctor will discuss these
with you in some detail before treatment starts. Any
possible side effects will depend upon the
particular treatment being used and, when
radiotherapy is being given, the part of the body
that is being treated. Side effects can include:
nausea (feeling sick) and vomiting, hair loss, an
increased risk of infection or bruising and
bleeding, tiredness constipation and diarrhoea.
Late side effects
A small number of children may develop side effects
many years after their treatment for a
rhabdomyosarcoma. Longer-term side effects depend on
the type of treatment used, and may include possible
reduced growth, infertility, a change in the way the
heart and the kidneys work, hearing problems and a
small increase in the risk of developing another
cancer in later life. If your child has received
daunorubicin (and anthracycline chemotherapy) then
your child may have a small risk of developing heart
problems. He/she will have been monitored throughout
treatment and following completion of this therapy.
Overall about two thirds of all children with
rhabdomyosarcoma are cured, but the particular risks
for your child will be discussed by your doctor.
After treatment the doctors will regularly check the
child to be sure the cancer has not come back and to
look for any long term side effects of the
treatment. After a while you will not need to visit
the clinic so often. If you have specific concerns
about your child's condition and treatment, it is
best to discuss them with your child's doctor, who
knows the situation in detail.
This definition includes different tumours with different
biology and natural history, some of which are more common
in adults. These tumours can arise, generally as a soft
part enlarging mass, anywhere in the body (most frequently
in the muscles of extremities, less usually in the trunk
or head and neck region). The cause of their origin is
unknown, and researchers are studying possible causes:
it is known that children with certain rare genetic
disorders, such as Li Fraumeni syndrome or neurofibromatosis,
have a higher risk of developing soft tissue sarcomas.
However, the majority of soft tissue sarcomas are sporadic.
Usually, non-rhabdomyosarcoma soft tissue sarcomas are
characterized by local aggressiveness. Metastases are
rare at the time of diagnosis; when they occur, they
are frequently localized at the lung. The propensity
to metastasize is directly correlated to the grade of
malignancy. Generally, low-grade tumours usually may
have local aggressiveness but low tendency to metastatic
spread. High-grade tumours have a more invasive behaviour
with higher propensity to metastasize. Overall, the majority
of patients with soft tissue sarcomas can be cured, even
if the probability of cure depends on the degree of malignancy
and the stage of the disease.
The most common sign that leads to the diagnosis of soft
tissue sarcomas is a growing swelling or lump. Other signs
and symptoms depend on the part of the body where the tumour
Various exams are necessary for the full diagnosis of soft
tissue sarcomas. First of all, biopsy is needed for defining
the histological diagnosis: a small sample of the tumour is
taken and looked under a microscope, so that the pathologist
can give the exact name of the tumour.
Then some tests are necessary to define the stage of the tumour
(size, local invasiveness, spread in other part of the body):
CT or MRI scan of the part of origin of the tumour, chest x-ray
and CT-scan, abdominal ultrasound, bone scan are generally
Knowing the particular type and the stage of the tumour is very
important for deciding the most appropriate treatment.
The treatment of patients with soft tissue sarcomas is complex
and may necessitate multidisciplinary approach, including
surgery, radiotherapy and chemotherapy. The treatment depends
upon the type of the tumour and its grade of malignancy, the
size of the tumour, and the possibility to remove it with a
Surgery is the mainstay of treatment. If possible, surgery
will be used to completely remove the tumour. If the tumour
cannot be completely removed without mutilation or functional
sequelae, radiotherapy and chemotherapy should be used to
shrink the tumour down and facilitate a subsequent operation.
Radiotherapy may be given to the area of origin of the tumour:
high-energy rays can destroy tumour cells, but it is important
to know that irradiation might cause late side effects
(i.e. possible reduced growth of the irradiated part) that
are difficult to foresee, but must be taken into account
when the treatment strategy is discussed.
Chemotherapy is a combination of drugs that can destroy tumour
cell with a cytotoxic mechanism. It is usually administered
every three weeks: given intravenously, it can act against
every tumour cell presents in the body, also against those
spreading away from the site of origin of the tumour.
Chemotherapy can be associated to nausea and vomiting,
hair loss and increased risk of infection. With the exception
of some particular histotypes, non-rhabdomyosarcoma soft
tissue sarcomas are generally considered tumours with uncertain
chemosensitiveness. But recent data would seem to suggest that
chemotherapy may play a more significant role than is generally
believed in some subsets of patients for which the risk of
failure is high.
Your doctors will explain you possible benefits and disvantages
of the treatment choices.