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Soft tissue sarcomas

JASCAP

JEET ASSOCIATION FOR SUPPORT TO CANCER PATIENTS, MUMBAI, INDIA

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JASCAP
JEET ASSOCIATION FOR SUPPORT TO CANCER PATIENTS
c/o. Abhay Bhagat & Co., Office No.4, “Shilpa”, 7th.Road, Prabhat Colony,
Santacruz (East), Mumbai – 400 055
Tel.: 2617 7543, 2616 0007. Fax: 91-22-2618 6162
E-mail :pkrjascap@gmail.com & abhay@abhaybhagat.com

JASCAP is a charitable trust that provides information on various aspects of cancer.

This can help the patient and his family to understand the disease and its treatment
and thus cope with it better.

Registered under the Societies Registration Act, 1860 No.1359 / 1996 G.B.B.S.D.,
Mumbai and under the Bombay Public Trusts Act, 1950 No. 18751 (Mumbai).
Donations to JASCAP qualify for deduction u/s 80G (1) of the Income Tax Act, 1961
vide Certificate No. DIT (E) / BC / 80G / 1383 / 96-97 dated 28.02.97 subsequently
renewed.

Contact: Mr. Prabhakar K. Rao or Mrs. Neera P. Rao

Publisher: JASCAP, Mumbai 400 055

Printer: Surekha Press, Mumbai 400 019

Edition: March 2011

Donation suggested Rs.25.00

o © Cancerbackup January 2009

o This booklet is an adaptation of “understanding cancer of the skin” produced by

Cancerbackup and is reproduced with their kind permission.

o JASCAP gratefully acknowledges Cancerbackup’s permission to reproduce this

booklet.
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Contents
General

 Soft tissue sarcomas

 What is cancer?
 Types of cancer
 Types of soft tissue sarcoma

Causes & diagnosis

 Risk factors and causes of Soft tissue sarcoma

 How common is the Soft tissue sarcoma in India?
 Symptoms
 Diagnosis
 Further tests
 Staging & grading

Treatment

 Treatment overview
 Surgery
 Limb-sparing surgery
 Amputation
 Radiotherapy
 Chemotherapy
 Biological therapies

After treatment

 Follow up
 Living with an amputation

Clinical trials

 Research - clinical trials

Types

 GIST **
 Leiomyosarcoma **
 Rhabdomyosarcoma **

Resources & support

 JASCAP resources

** JASCAP has factsheets on each of these types of soft tissue sarcomas.

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What are soft tissue sarcomas?

Soft tissue sarcomas are rare. About 2200 people a year in the UK will be diagnosed
with a sarcoma.

Soft tissue sarcomas are cancers that develop from cells in the soft, supporting
tissues of the body. They can occur in muscle, fat, blood vessels or in any of the
other tissues that support, surround and protect the organs of the body. Soft tissue
sarcomas can also develop in specific organs, such as the womb (uterus), stomach,
skin and small bowel.

Some types of sarcoma occur in children, teenagers and young adults, but generally
sarcomas are more likely to develop in people over the age of 30.

Almost half of all soft tissue sarcomas occur in the limbs – especially the legs. Other
common sites are the chest, abdomen and pelvis. Less commonly they may occur in
the head and neck.

Bone sarcomas

Some sarcomas, such as osteosarcomas, start in the bone. These grow and develop
differently and are treated differently from soft tissue sarcomas. Occasionally it is
hard to tell whether a sarcoma has started in soft tissue or bone. There are some
types of sarcoma, such as Ewing’s tumours, that can begin in either bone or soft
tissue.

We have a separate section on cancer that starts in the bone; primary bone cancer.

What is cancer?
The organs and tissues of the body are made up of tiny building blocks called cells.
Cancer is a disease of these cells.

Cells in different parts of the body may look and work differently but most reproduce
themselves in the same way. Cells are constantly becoming old and dying, and new
cells are produced to replace them. Normally, cells divide in an orderly and controlled
manner. If for some reason the process gets out of control, the cells carry on dividing,
developing into a lump which is called a tumour.
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Tumours can be either benign or malignant. Cancer is the name given to a

malignant tumour. Doctors can tell if a tumour is benign or malignant by examining a
small sample of cells under a microscope. This is called a biopsy.

In a benign tumour the cells do not spread to other parts of the body and so are not
cancerous. However, if they continue to grow at the original site, they may cause a
problem by pressing on the surrounding organs.

A malignant tumour consists of cancer cells that have the ability to spread beyond
the original area. If the tumour is left untreated, it may spread into and destroy
surrounding tissue. Sometimes cells break away from the original (primary) cancer.
They may spread to other organs in the body through the bloodstream or lymphatic
system.

The lymphatic system is part of the immune system - the body's natural defence
against infection and disease. It is a complex system made up of organs, such as
bone marrow, the thymus, the spleen, and lymph nodes. The lymph nodes (or
glands) throughout the body are connected by a network of tiny lymphatic ducts.

When the cancer cells reach a new area they may go on dividing and form a new
tumour. This is known as a secondary cancer or metastasis.

It is important to realise that cancer is not a single disease with a single type of
treatment. There are more than 200 different kinds of cancer, each with its own name
and treatment.

Types of cancer
Carcinomas

The majority of cancers, about 85% (85 in a 100), are carcinomas. They start in the
epithelium, which is the covering (or lining) of organs and of the body (the skin). The
common forms of breast, lung, prostate and bowel cancer are all carcinomas.

Carcinomas are named after the type of epithelial cell that they started in and the part
of the body that is affected. There are four different types of epithelial cells:

squamous cells - that line different parts of the body, such as the mouth,
gullet (oesophagus), and the airways
adeno cells - form the lining of all the glands in the body and can be found in
organs such as the stomach, ovaries, kidneys and prostate
transitional cells - are only found in the lining of the bladder and parts of the
urinary system
basal cells - that are found in one of the layers of the skin.

A cancer that starts in squamous cells is called a squamous cell carcinoma. A cancer
that starts in glandular cells is called an adenocarcinoma. Cancers that start in
transitional cells are transitional cell carcinomas, and those that start in basal cells
are basal cell carcinomas.
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Leukaemias and lymphomas

These occur in the tissues where white blood cells (which fight infection in the body)
are formed, i.e. the bone marrow and lymphatic system. Leukaemia and lymphoma
are quite rare and make up about 6.5% (6.5 in 100) of all cancers.

Sarcomas

Sarcomas are very rare. They are a group of cancers that form in the connective or
supportive tissues of the body such as muscle, bone and fatty tissue. They account
for less than 1% (1 in 100) of cancers.

Sarcomas are split into two main types:

bone sarcomas - that are found in the bones

soft tissue sarcomas - that develop in the other supportive tissues of the
body.

Others forms of cancer

Brain tumours and other very rare forms of cancer make up the remainder of
cancers.

Types of soft tissue sarcoma

There are many types of soft tissue sarcoma, and they tend to grow and develop
differently. The most common types are described below. Your doctor will be able to
give you more details about which type of sarcoma you have. Each type of sarcoma
is named after the type of cell from which it has grown, rather than the part of the
body in which it started.

Fibrosarcomas
Myxofibrosarcomas
Desmoid tumours
Liposarcomas
Synovial sarcomas
Rhabdomyosarcomas
Leiomyosarcomas
Malignant peripheral nerve sheath tumours (MPNST)
Angiosarcomas
Gastrointestinal stromal tumours (GIST)
Kaposi's sarcoma (KS)
Other sarcomas
Ewing's tumours
Soft tissue sarcomas in children

Fibrosarcomas

These sarcomas start in cells called fibrocytes, which make up the fibrous tissues
that join together the inner structures of the body: for example, muscles to bones.
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They are most commonly found on the arms, legs or trunk, but can occur deeper in
the body. Most people first notice them as a painless, firm lump.

Myxofibrosarcomas

This is a type of fibrous sarcoma and the most common type of sarcoma in older
people. They were previously called malignant fibrous histiocytomas (MFH). It is not
clear which type of cell they start from. Myxofibrosarcomas can affect any part of the
body, but most commonly occur in the arms or legs.

Desmoid tumours

These tumours are another type of fibrous sarcoma. They are slow-growing and are
sometimes said to be halfway between a fibrosarcoma and a benign fibroma (a non-
cancerous tumour of fibrous tissue). Desmoid tumours do not tend to spread to other
parts of the body, but can spread into nearby tissues and so are usually treated in a
similar way to sarcomas.

Liposarcomas

These sarcomas start in the body’s fat cells. They can grow anywhere in the body
and most commonly affect middle-aged people. Some grow very slowly (taking many
years to develop) and others more quickly.

Synovial sarcomas

Synovial sarcomas usually start near to joints, such as the knee or elbow, but can
occur in any part of the body. They usually appear as hard lumps and are more
common in younger adults.

Rhabdomyosarcomas

Rhabdomyosarcomas grow in the active muscles of the body that we can control.
These muscles are known as skeletal muscle or striated muscle.
Rhabdomyosarcomas occur mostly in the head, neck and pelvis, but can occur in the
arms or legs. There are three sub-types of rhabdomyosarcoma: embryonal, alveolar
and pleomorphic.

Embryonal rhabdomyosarcomas tend to occur more commonly in children, while

alveolar rhabdomyosarcomas occur more in the limbs of teenagers and young adults.
Pleomorphic rhabdomyosarcoma tends to occur in middle-aged people.

Leiomyosarcomas

Leiomyosarcomas start from smooth muscle that is not under our conscious control.
Smooth muscle is also called involuntary muscle and forms the walls of the womb,
stomach, intestine and the blood vessels. Leiomyosarcoma is one of the more
common types of sarcoma and can occur anywhere in the body.
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Malignant peripheral nerve sheath tumours (MPNST)

These sarcomas arise in the cells that cover nerve cells and can occur anywhere in
the body. The cells around the nerve cells are called schwann cells. MPNST can also
be called malignant schwannomas or neurofibrosarcomas. They most commonly
occur in people who have a rare genetic disorder called neurofibromatosis (von
Recklinghausen’s disease).

Angiosarcomas

Angiosarcomas start from the cells that make up the walls of blood or lymph vessels.
If they develop from blood vessels they are called haemangiosarcomas. If they start
from the lymph vessels they are called lymphangiosarcomas. Angiosarcomas
sometimes arise in a part of the body that has been treated with radiotherapy many
years before.

Gastrointestinal stromal tumours (GIST)

GISTs are sarcomas that develop from the connective tissues in the walls of the
digestive system. The digestive system is often called the gastrointestinal (GI) tract.
This type of tumour may also be called GI stromal sarcoma. They behave differently
from other types of sarcoma and are treated very differently.

Kaposi's sarcoma (KS)

Although Kaposi’s sarcoma is a type of sarcoma, it differs from other sarcomas in the
way it develops. It starts from cells in the skin. Coloured patches or lumps can
develop in the skin, in the mouth, and in the lymph nodes or internal organs such as
the lung, liver or spleen.

Kaposi’s sarcoma can affect people with a weakened immune system, including
people with HIV and Aids. Other types can affect people of Jewish, Italian and West
African origin. Kaposi’s sarcoma is treated differently to other types of soft tissue
sarcoma.

Other sarcomas

There are other, much rarer, types of sarcoma. These include:

alveolar soft part sarcoma

dermatofibrosarcoma protuberans (DFSP)
desmoplastic small round cell tumours
epithelioid sarcomas
extraskeletal myxoid chondrosarcomas
giant cell fibroblastoma (GCF).
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Ewing's tumours

Ewing’s tumours are a type of bone sarcoma, but about a third of all Ewing’s tumours
develop in the soft tissue and are known as extra-osseous Ewing’s tumours. Soft
tissue Ewing’s sarcomas tend to behave differently to other soft tissue sarcomas and
are usually treated in a similar way to bone sarcomas.

Soft tissue sarcomas in children

Soft tissue sarcomas also occur in children, particularly some types of

rhabdomyosarcoma. Their symptoms and treatment may differ from those in an adult
with the same sarcoma.

Our section on children's cancer discusses the treatment of children's cancers and
includes information about some types of sarcoma that occur in children.

Risk factors and causes of soft tissue sarcoma

Although the cause of soft tissue sarcoma is unknown, research into this is ongoing.
Sarcomas, like other cancers, are not infectious and can’t be passed on to other
people.

Age

Sarcomas can occur at any age but are more common in people over 30.

Genetic conditions

Most sarcomas are not caused by an inherited faulty gene that can be passed on to
other family members. Members of your family are not likely to have an increased
risk of developing a soft tissue sarcoma just because you have.

However, people who have some rare inherited genetic conditions are more at risk of
developing a sarcoma. These conditions include neurofibromatosis, Gardner
syndrome, Li-Fraumeni syndrome and retinoblastoma. You would normally know if
any member of your family had one of these conditions, and their doctor would check
them regularly for any sign of a sarcoma.

Previous radiotherapy treatment

Very rarely, soft tissue sarcomas may occur in a part of the body that has previously
been treated with radiotherapy for another type of cancer. The sarcoma will not
usually develop until at least 5–10 years after the radiotherapy treatment.
Improvements in giving radiotherapy treatment mean that the risk of developing a
sarcoma is very small.
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Exposure to chemicals

The development of some sarcomas may be linked to exposure to some types of

chemicals. The chemicals include: vinyl chloride, which is used for making plastics;
some types of herbicides (weedkillers); and dioxins, which are a waste product
produced during the manufacture of chemicals and fertilisers.

Injury

There is no evidence that an injury can cause a soft tissue sarcoma to develop. It’s
possible that an injury may draw attention to a sarcoma that was already there and
not causing any symptoms, but the sarcoma will have taken many years to develop.

How common is the Soft tissue sarcoma in India?

Connective tissue and soft tissue sarcoma is one of the rare cancers diagnosed in
India. It ranks lower than number 15 among all other cancers for people from the
Indian subcontinent1.

In India, between the years 2001-2003, across five urban centers - Mumbai, Delhi,
Chennai, Bhopal and Bangalore, – and one rural center - Barshi, a total of 678 cases
of connective tissue and soft tissue sarcoma were registered (1.54% of all cancers)
for males across all age groups; while 501 cases of connective tissue and soft tissue
sarcoma were registered (1.12% of all cancers) for females across all age groups.
Considering all men, women and children with all types of cancers together, a grand
total of 1,179 cases of connective tissue and soft tissue sarcoma (1.33% of all
cancers) were registered at the six centers mentioned above, between the year
2001-20032.

The TATA Memorial Hospital (T.M.H.) in Mumbai, India registered a grand-total of

19,127 cases of all types of cancer patients in the year 2006 for men, women and
children combined, out of which 372 (1.94% of the total cases) were diagnosed with
the soft tissue sarcoma. Out of the total 372 patients diagnosed with soft tissue
sarcoma, mentioned above at the T.M.H., 251 (68%) were males and 121 (32%)
were females3.

1
Globocan 2008: Cancer incidence and mortality rates worldwide

2
Population based cancer registry 2001-2003 Mumbai, Delhi, Chennai, Bhopal, Barshi and Banglore,
Indian Cancer Society.

3
TATA Memorial Hospital Registry Data for 2006
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Symptoms of soft tissue sarcomas

Sarcomas often don’t cause any symptoms for a long time. They can start in any part
of the body and the symptoms will depend on the part of the body that is affected.

If the sarcoma is in an arm or a leg, the most common symptom is an uncomfortable

swelling in the affected limb. Occasionally, this swelling may be painful or tender, but
it may also be painless.

If the sarcoma is in the central part of the body (the trunk), the symptoms will depend
on which organ in the body is affected. For example:

a sarcoma in a lung may cause a cough and breathlessness

a sarcoma in the abdomen could cause abdominal pain, vomiting and
constipation
a sarcoma affecting the womb may cause bleeding from the vagina and pain
in the lower part of the abdomen.

Generally, soft tissue sarcomas do not cause any symptoms until they are quite large
and are pressing on an organ in the body or on a nerve or muscle. Things to look out
for include:

any lump, especially if it is increasing in size and is bigger than 5cm (2in)
any lump that is painful or tender
any lump that is deep in the body (ie not just under the skin)
any lump that has come back after being surgically removed.

If you notice any of the above, contact your GP; but remember that these symptoms
can also be caused by other conditions.

How soft tissue sarcomas are diagnosed

Usually you begin by seeing your GP, who will ask you about your symptoms and
examine you. They may arrange for you to have any tests or x-rays that are
necessary. You may need to be referred to hospital for these tests and for specialist
advice and treatment. If your GP suspects that you might have a sarcoma you will
usually be seen at the hospital within two weeks.

At the hospital, the specialist will ask about your general health and any previous
medical problems. They will also examine you, which will include feeling the area
where there is pain or swelling. You may be asked to have blood tests and a chest x-
ray to check your general health.

You are also likely to be asked for your permission to take a sample of the lump to
examine under the microscope. This is known as a biopsy and is the only way to tell
whether the lump is a cancer or a non-cancerous (benign) tumour. You may have
other tests and scans to assess the lump, before a biopsy.
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A biopsy can be done in either of the following ways:

Core needle biopsy

Surgical biopsy

Core needle biopsy

A needle is put into the lump to take a sample of cells. Several samples may be
taken. A local anaesthetic is injected first to numb the area. If the lump is near the
surface of your body and can easily be felt, the doctor will probably just feel it to
guide the needle in. If the lump is deep within the body (such as in the abdomen) or
is harder to feel, the doctor will use an ultrasound scan or sometimes a CT scan to
see where the needle is going and guide it into the right place.

When the cells are looked at under a microscope, the pathologist will be able to tell
whether they are benign (not cancerous) or cancerous cells. If the lump is a sarcoma,
further tests may be done on the sample to try to find out exactly what type of
sarcoma it is.

Sometimes, particularly with children, the biopsy is done under a general

anaesthetic.

For most people a needle biopsy will show whether the lump is a sarcoma.
Sometimes, not enough cells are collected to give a clear answer, and then a
surgical biopsy is needed.

Surgical biopsy

This will only be done if a needle biopsy can’t be used, or doesn’t give a definite
result. Surgical biopsy means using a surgical knife (scalpel) to open the area and
remove a tissue sample from the lump. If the lump is small enough, the whole of it
may be removed.

A surgical biopsy may be done under a local or general anaesthetic, depending on

the position of the lump and how deep it is within the body. If the lump turns out to be
benign, you may not need to have any more treatment. If it is cancer, your doctor will
discuss the treatment options with you.

In the same way as with a needle biopsy, the sample will be sent to the laboratory so
that it can be tested. Often a large number of studies will be done even on a very
small sample. It can take from a few days to 10 days to get all the results. This can
be a worrying time for you, but it is very important that an accurate diagnosis is made
so that the most appropriate treatment can be given.

It may help you to talk about your worries with a partner, close friend, relative or
counsellor (see the list of useful organisations).
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Further tests for soft tissue sarcomas

Your doctor may arrange for you to have one or more of the following tests to find out
the size of the sarcoma, exactly where it is and whether or not it has spread to other
parts of the body.

Chest x-ray
CT scan
MRI scan
Ultrasound scan of the abdomen
PET scan
Waiting for your test results

Chest x-ray

This will be done to check your general health and to look for any sign that the
cancer has spread to your lungs, as this is one of the commonest places for soft
tissue sarcomas to spread to.

CT scan

A CT (computerised tomography) scan takes a series of x-rays which builds up a

three-dimensional picture of the inside of the body. The scan is painless but takes
from 10 to 30 minutes. CT scans use a small amount of radiation, which will be very
unlikely to harm you and will not harm anyone you come into contact with. You will be
asked not to eat or drink for at least four hours before the scan.

Having a CT scan

You may be given a drink or injection of a dye which allows particular areas to be
seen more clearly. For a few minutes, this may make you feel hot all over. If you are
allergic to iodine or have asthma you could have a more serious reaction to the
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injection, so it is important to let your doctor know beforehand. You will probably be
able to go home shortly after the scan is over.

MRI scan

An MRI (magnetic resonance imaging) scan is similar to a CT scan, but uses

magnetism instead of X-rays to build up cross-sectional pictures of your body.

During the test you will be asked to lie very still on a couch inside a long tube for
about 30 minutes. This is painless but can be slightly uncomfortable, and some
people feel a bit claustrophobic during the scan. It is also noisy, but you will be given
earplugs or headphones. You can usually take someone with you into the room to
keep you company. It is not possible for you to have an MRI scan if you have any
metal in your body such as a pacemaker, or surgical clips.

Some people are given an injection of dye into a vein in the arm, but this usually
does not cause any discomfort. You will probably be able to go home when the scan
is over.

Ultrasound scan of the abdomen

This may be used if you have an abdominal lump. An ultrasound scan uses sound
waves to look at internal organs such as the liver and the inside of the abdomen. You
will usually be asked not to eat or to drink for a few hours before the test.

Once you are lying comfortably on your back, a gel is spread onto your abdomen. A
small device like a microphone, which produces sound waves, is passed over the
area. The sound waves are then converted into a picture by a computer. The test
only takes a few minutes.

If the lump is in your womb, the ultrasound scan may be done by inserting an
ultrasound device into your vagina. This is known as a transvaginal ultrasound scan
and gives a very clear picture of the womb.

Having an ultrasound scan

PET scan

PET (positron emission tomography) scans are a newer type of scan and you may
have to travel to a specialist centre to have one. They are not always necessary but
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you can discuss with your doctor whether one would be useful in your case. PET
scans can be used to find if a sarcoma has spread to other parts of the body, or to
examine any lumps that remain after treatment, to see if these are scar tissue or if
cancer cells are still present.

A PET scan uses low-dose radioactive glucose (a type of sugar) to measure the
activity of cells in different parts of the body. A very small amount of a mildly
radioactive substance is injected into a vein, usually in your arm. A scan is then taken
a couple of hours later. Areas of cancer are usually more active than surrounding
tissue and show up on the scan.

Waiting for your test results

It will probably take several days for the results of your tests to be ready and this
waiting period will obviously be an anxious time for you. It may help if you can talk
things over with a relative or close friend. You may wish to ring Cancerbackup or
another support organisation for emotional support.

Staging and grading soft tissue sarcomas

The further tests already described help to find the stage and the grade of the
cancer. These help the doctors to decide on the most appropriate treatment.

Grading
Staging

Grading

Grading refers to the appearance of the cancer cells under the microscope. The
grade gives an idea of how quickly the cancer may develop. Grading of soft tissue
sarcomas can sometimes be difficult, especially for the less common types. There
are four grades, from 1 to 4.

Grade 1 means that the cancer cells look very like the normal cells of the soft
tissues (called well-differentiated). They are usually slow-growing and are
less likely to spread.
Grade 2 means the cancer cells have begun to look more abnormal. They are
described as being moderately differentiated.
Grade 3 soft tissue sarcomas are described as being poorly differentiated –
the cancer cells look very abnormal, are likely to grow quickly, and are more
likely to spread.
Grade 4 soft tissue sarcomas are either poorly differentiated or
undifferentiated. Undifferentiated cancer cells are primitive and immature.
They don’t function properly and have no specific appearance. Grade 4 soft
tissue sarcomas are likely to grow more quickly and are more likely to spread.
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Staging

The stage of a cancer describes its size and whether it has spread beyond its original
area of the body.

Several different staging systems may be used for soft tissue sarcomas. A commonly
used staging system, produced by the American Joint Committee on Cancer (AJCC),
is described below and includes information about the grade of the cancer as well as
the stage:

There are four major stages (from 1 to 4).

Stage 1 The cancer is low-grade (grades 1 or 2) and can either be close to

the surface of the body (superficial) or deep within the body. There is no sign
of any spread.
Stage 2 The cancer is high grade (grades 3 or 4). It is either superficial or
deep within the body, but has not spread to lymph nodes or other parts of the
body.
Stage 3 The cancer is described as high-grade, large (5cm or bigger) and
deep. It has not begun to spread.
Stage 4 The cancer can be any grade, but it has spread to lymph nodes in
the area or to any other part of the body, such as the lungs, head or neck.
This is known as secondary cancer (or metastatic cancer).

Recurrence means that a soft tissue sarcoma has come back after it was first
treated. It may come back in the area where it first started, or it may come back in
another part of the body.

TNM staging system

Another staging system known as the TNM system is also commonly used. This can
give your doctors more precise information about the extent of the cancer.

T describes the size of the tumour.

N describes whether the cancer has spread to the lymph nodes.
M describes whether the cancer has spread to another part of the body
(secondary or metastatic cancer).

Treatment for soft tissue sarcomas

Treatment planning
Types of treatment
Second opinion
Giving your consent
Benefits and disadvantages of treatment
Treatment decisions
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Treatment planning

As sarcomas are rare cancers, you should always be referred for treatment at a
specialist sarcoma unit where a team of specialist doctors and others work together.
This is known as a multidisciplinary team (MDT) and may include:

a surgeon
a clinical oncologist – a doctor who treats cancer with radiotherapy
a medical oncologist – a doctor who treats cancer with chemotherapy
a pathologist – a doctor who specialises in how disease affects the body
a radiologist – a doctor who analyses x-rays and scans
a specialist nurse who gives information and support to people with sarcoma.

The multidisciplinary team may also include other healthcare professionals, such as:

a dietitian
physiotherapist
occupational therapist
psychologist or counsellor.

In order to plan the best treatment for you, your doctors will take into account your
age, general health, and the type, stage and grade of the sarcoma.

Types of treatment

Surgery is the most common treatment. If the soft tissue sarcoma is small and it is
possible to remove it completely, surgery may be used on its own.

For larger sarcomas, and where there may be a possibility of cancer cells being left
behind, radiotherapy is usually used as well as surgery. Radiotherapy may be given
before the operation to shrink the tumour and make it easier to remove, or afterwards
to try to destroy any cancer cells that may not have been removed.

Sometimes radiotherapy is used on its own or in combination with chemotherapy to

treat more advanced sarcomas that can’t be removed.

Chemotherapy may sometimes be given before surgery, to shrink the tumour. It is

sometimes given after surgery, to try to destroy any cancer cells that have not been
removed or which may have spread to other parts of the body.

For gastrointestinal stromal tumours a drug called imatinib (Glivec®) may be used
before or after surgery.

It often helps to make a list of the questions you want to ask your doctor, and to take
a close friend or relative with you.

Second opinion

Usually a number of cancer specialists work together as a team and they use
national treatment guidelines to decide on the most suitable treatment for a patient.
Even so, you may want to have another medical opinion. Either your specialist, or
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your GP, will be able to refer you to another specialist for a second opinion if you feel
it will be helpful. The second opinion may cause a delay to the start of your
treatment, so you and your doctor need to be confident that it will give you useful
information.

If you do go for a second opinion, it may be a good idea to take a friend or relative
with you, and have a list of questions ready, so that you can make sure your
concerns are covered during the discussion.

Giving your consent

Before you have any treatment, your doctor will explain the aims of the treatment to
you. They will usually ask you to sign a form saying that you give your permission
(consent) for the hospital staff to give you the treatment. No medical treatment can
be given without your consent, and before you are asked to sign the form, you should
have been given full information about:

the type and extent of the treatment you are advised to have
the advantages and disadvantages of the treatment
any other types of treatments that may be appropriate
any significant risks or side effects of the treatment.

If you do not understand what you have been told, let the staff know straight away so
that they can explain again. Some cancer treatments can be very complex, so it is
not unusual for people to need repeated explanations.

It is often a good idea to have a friend or relative with you when the treatment is
explained, to help you remember the discussion more fully. You may also find it
useful to write down a list of questions before you go to your appointment.

Patients often feel that the hospital staff are too busy to answer their questions, but it
is important for you to be aware of how the treatment is likely to affect you. The staff
should be willing to make time for you to ask questions.

You can always ask for more time to decide about the treatment if you feel that you
can’t make a decision when it is first explained to you.

You are also free to choose not to have the treatment. The staff can explain what
may happen if you do not have it. It is essential to tell a doctor, or the nurse in
charge, so that they can record your decision in your medical notes. You do not have
to give a reason for not wanting to have treatment, but it can be helpful to let the staff
know your concerns so that they can give you the best advice.

Benefits and disadvantages of treatment

Many people are frightened at the idea of having cancer treatments, because of the
potential side effects that can occur. Although many of the treatments can cause side
effects, these can often be well controlled with medicines. Some people ask what
would happen if they did not have any treatment. Treatment can be given for different
reasons and the potential benefits will vary depending upon the individual situation.
 19

Early-stage sarcoma

In people with early-stage sarcoma, surgery is often done with the aim of curing the
cancer, and additional treatments may also be given to reduce the risks of it coming
back.

Advanced-stage sarcoma (metastatic)

If the cancer is at a more advanced stage, the treatment may only be able to control
it – leading to an improvement in symptoms and a better quality of life. However, for
some people the treatment will have no effect upon the cancer and they will get the
side effects with little benefit.

Treatment decisions

If you have been offered treatment that is intended to cure your cancer, the decision
whether to accept it may not be a difficult one. However, if a cure is not possible and
the treatment is being given to control the cancer for a period of time, it may be more
difficult to decide whether to go ahead with treatment or not.

Making decisions about treatment in these circumstances is always difficult, and you
may need to discuss in detail with your doctor whether you wish to have treatment. If
you choose not to, you can still be given supportive (palliative) care, with medicines
to control any symptoms.

It is important that you ask your doctors and nurses any questions you have about
your treatment. It is true that the hospital staff are busy, but the more you understand
about your treatment, the easier it is for you and them.

Surgery for soft tissue sarcomas

Soft tissue sarcomas are very rare cancers, so once a sarcoma is diagnosed you will
usually be referred to a surgeon in a large cancer hospital, who specialises in the
treatment of these types of cancer. Sometimes surgery may have been done before
the surgeon realised that the lump was a soft tissue sarcoma. In this situation you will
then normally be referred to a sarcoma specialist. Sarcoma specialists often treat
other types of cancer as well as sarcoma.

The aim of most sarcoma surgery is to remove as much of the tumour as possible.
Usually, an operation called a wide local excision is done. This means removing the
cancer with a border (margin) of healthy, cancer-free tissue all around it. The border
of healthy tissue is removed to try to stop the cancer coming back in that area.

It is difficult to give general information about sarcoma surgery because the type of
operation you have will depend on where in your body the sarcoma is. Many
sarcomas are in the arm or leg, but they can grow anywhere.

Your surgeon will discuss the operation with you in detail before any choice is made
about your treatment.
 20

Surgery to the chest or abdomen

Surgery to the arms or legs
Plastic surgery

Surgery to the chest or abdomen

Surgery is usually the main treatment for soft tissue sarcomas of the trunk (the chest
and abdomen). The extent of the operation will depend on the exact position of the
sarcoma in the body. Your doctor will discuss this fully with you before the operation.

After your operation, you will have a tube (drip) going into a vein in your arm. This will
give you fluids and nutrients for 2–3 days until you are able to eat and drink properly.
The nurses on the ward will then take the drip out. Your doctor will let you know when
you can start to eat again.

Radiotherapy may also be used after operations for sarcomas in the chest or
abdomen to try to make sure that any remaining cancer cells are destroyed.

Surgery to the arms or legs

Major improvements have been made in surgery for soft tissue sarcomas of the arms
and legs over the past few years. In the past, it was usually necessary to remove the
limb (amputation) if cancer was found. A false limb (prosthesis) would then be made
to replace the limb that had been removed. Amputation is still sometimes necessary,
but it is now usually possible to just remove the cancer and some of the tissue
around it. This is known as limb-sparing surgery and is done by using a combination
of surgery, radiotherapy and chemotherapy.

Plastic surgery

Depending on the size and position of your tumour, the surgeon may have to remove
a large area of tissue. This may mean that you need to have some
reconstructive/cosmetic (plastic) surgery to repair the area. If this kind of surgery is
needed it will be done at the same time as your operation.

The aim of plastic surgery is to make the area look and function as naturally as
possible. The exact type of surgery you have will depend on where in your body the
sarcoma is and how much tissue the surgeon needs to remove. If only a small area
has been removed, the surgeon may be able to join the two edges together again.
For larger areas a skin graft may be needed. This involves taking some skin from a
different part of the body (known as the donor site) and using it to repair the
operation site. Both sites will gradually heal over a few weeks after the operation.

Occasionally it’s necessary to repair the operation site with a specialist technique
using skin and tissue. This is known as a tissue flap and there are several different
types. The surgeon will explain your operation to you and answer any questions you
may have.
 21

Limb-sparing surgery for soft tissue sarcomas

Before your operation
After your operation
Radiotherapy
Prostheses for growing children

Before your operation

Before your operation, your doctor and the nurses on the ward will discuss your
treatment with you in detail to make sure that you fully understand what it involves. It
is often helpful to talk to someone who has had the same operation, and the medical
staff should be able to arrange this for you. On some wards a clinical nurse specialist
may be available to discuss any worries that you or your carers may have.

If you have had chemotherapy, your body will need some time to recover before the
surgery can be done. Usually it takes at least a couple of weeks after chemotherapy
before you are ready to have your operation.

Your doctor and anaesthetist will come to see you to make sure you understand what
is going to happen. They will answer any questions you may have. You will have to
sign a form agreeing to the surgery. This is the time to make sure you ask all the
questions you need to. Many people find that the more they know about what is
going to happen, the less frightening it seems. Don’t worry if you think of more
questions later; just speak to your nurses again. If they can’t answer your questions,
they can contact the doctor to come and talk to you again.

If you have body hair on the surgery area, you will need to be shaved before your
operation. This is done to reduce the risk of infection. You may be shaved in the
operating theatre after you have had your anaesthetic.

After your operation

At first your limb will be firmly bandaged. This is to give the area time to heal. You will
probably have a drainage tube in the wound to remove any fluid that collects in the
area of the operation. It will be removed once it has stopped draining, usually after a
few days.

Swelling around the wound

Sometimes fluid can build-up around the wound, especially following surgery to a
buttock or limb. The swelling should gradually reduce over a few weeks. Sometimes
a lot of fluid builds up around the wound; this is known as a seroma. It may need to
be drained by a doctor or nurse.

Nutrition

You may have a drip (infusion) of fluids going into a vein in your arm. This will give
you fluids and nutrients for 2–3 days until you are able to eat and drink properly. The
nurses on the ward will then take the drip out.
 22

Pain

You will have some pain and discomfort after your operation. Painkillers will be
prescribed for you and these are usually very effective in keeping pain under control.
To start with you will probably need a strong painkiller such as morphine. This will be
given to you either as injections (given by the nurses), or through a small pump
attached to a needle in your arm (which you control yourself). It is important to let the
nurses know if your painkillers don’t seem to be working.

If you have had surgery on your leg, a different method of pain relief (called an
epidural) may be used. A fine tube is inserted through your back into the fluid
between the membranes around your spinal cord; a local anaesthetic can then be
continuously given into the fluid to numb the nerves that run to your legs.

Exercises

Your nurses or the physiotherapist will teach you breathing and leg exercises. You
can help yourself to get better by doing the exercises as often as you are told you
need to. Breathing exercises will help to stop you getting a chest infection. Leg
exercises will help to stop clots forming in your legs. Chest infections or blood clots
can happen if you are not moving around as much as you would normally be. Your
nurses will encourage you to get up and about as soon as possible. However, limb-
sparing surgery or amputation is major surgery, and you may have to stay in bed for
some time afterwards; this may be from a couple of days up to a week.

Your physiotherapist will show you some exercises to keep the muscles in the limb
strong and supple. This is so that as soon as it is strong enough you can use it
normally. The physiotherapist will also help you to move the limb once you are able
to be up and about again. Sometimes you will need a brace or support for your limb.
If the operation was on your leg, you may be given crutches to use at first. This is to
protect your leg while you are learning to walk again. You will probably be left with a
slight limp.

Sometimes trying to walk normally with a limp puts pressure on the leg and causes
pain elsewhere in the body. Although it is important to have any continuing pain
checked by your doctor, it does not necessarily mean that the cancer has come back.

Going home

Most people are able to go home once their wound is well healed, usually from 7–10
days after their operation.

Radiotherapy

After limb-sparing surgery, radiotherapy treatment is usually given to the area of the
operation, to destroy any cancer cells that may still be in the area. This is done
because it is very difficult to be completely certain that all the cancer cells have been
removed during the operation.
 23

Prostheses for growing children

If a child has limb-sparing surgery while they are still growing it may be possible for
them to have an internal prosthesis (false part of the limb) fitted that can be
lengthened at a later date. The lengthening is done during a fairly minor operation,
which may mean a short stay in hospital.

Amputation as a treatment for soft tissue sarcomas

It is not always possible to do limb-sparing surgery and occasionally amputation may
be necessary. This may be because it’s the only way to get rid of the cancer. Very
occasionally, after discussion with their specialist doctor and family, people choose to
have an amputation instead of limb-sparing surgery.

The preparation for amputation is similar to that for limb-sparing surgery.

Psychological support for people who are about to have an amputation is very
important. The nursing and medical staff looking after you will be able to offer help
and support. It may also be helpful to talk to someone who has had the same
operation and can give you practical advice.

After your operation

Artificial limb (prosthesis)

After your operation

You will usually have a drip for a few days to give you fluids. A bandage will be
applied over the affected site to help shape the area. You'll have a tube in the wound
to drain off any fluid that builds up.

You will be given painkillers to deal with any pain and discomfort. Some people have
a pain that appears to come from the part of the limb that has been amputated. This
is known as phantom pain or sensation. Although this pain will gradually fade, there
may be some discomfort in the area for a while after the operation.

About two to three days after surgery, you will be encouraged and helped to move
around. The physiotherapist will visit you shortly after your operation and show you
how to do exercises to keep the muscles around the operation site strong and
supple, making it easier to use an artificial limb. The physiotherapist will also show
you how to do the breathing and leg exercises described above.

Artificial limb (prosthesis)

Following an amputation, most people can be fitted with an artificial limb called a
prosthesis. Modern technology means that artificial limbs are now very effective,
enabling people to walk, run and play sport.

A person who fits artificial limbs will visit you before or after your operation to show
you the different types of prosthesis and how they work. Careful measurements have
to be taken so that the prosthesis fits properly. It may take several weeks for your
 24

prosthesis to be made and, in the meantime, you may be fitted with a temporary one
so that you can begin to get used to it. The fitting of artificial limbs is usually arranged
through the ward where you are being treated. Your doctor or nurse can give you
more information about this.

Once your wound has healed, probably after a couple of weeks, you will be able to
go home.

Radiotherapy for soft tissue sarcomas

About radiotherapy
Planning your treatment
Treatment sessions
Side effects

About radiotherapy

Radiotherapy treats cancer by using high-energy x-rays to destroy cancer cells, while
doing as little harm as possible to normal cells.

Radiotherapy is an important part of the treatment for soft tissue sarcomas,

particularly those affecting the arms or legs. It is usually given after surgery to
destroy any remaining cancer cells. Sometimes radiotherapy may be given before an
operation (neo-adjuvant radiotherapy), either to shrink the tumour and make it
easier for the surgeon to remove it successfully, or to avoid having to amputate the
limb. If the radiotherapy is given after surgery, the wound from the operation must be
healed before radiotherapy treatment is started.

Radiotherapy is sometimes given on its own and may be the only treatment needed.
Radiotherapy is also given to relieve symptoms and control sarcomas that can’t be
removed.

Radiotherapy for sarcomas is normally given as a series of short daily treatments in

the hospital radiotherapy department. High-energy x-rays are directed from a
machine at the area of the cancer. The treatments are usually given from Monday to
Friday, with a rest at the weekend. The number of treatments will depend on the
type, size and position of the cancer within your body, but the whole course of
treatment for early cancer will usually last a few weeks. Each treatment takes about
10–15 minutes. Your doctor will discuss the treatment and possible side effects with
you.

Planning your treatment

To make sure that the radiotherapy is as effective as possible, it has to be planned

carefully. If your sarcoma is in an arm or a leg, you may have a plastic mould made.
The mould helps to keep you still during radiotherapy treatment sessions and is fitted
over the affected part of your body. It is made in the mould room, on your first few
visits to the radiotherapy department.
 25

You will also be asked to lie under a large machine called a simulator, which takes x-
rays or CT scans of the area to be treated. Sometimes a CT scanner can be used for
the same purpose. Treatment planning is an essential element of radiotherapy and it
may take a few visits. The planning sessions during your first few visits will take
longer than the actual radiotherapy treatment sessions.

Marks may be drawn on your skin to help the radiographer, who gives you your
treatment, to position you accurately and to show where to direct the rays. These
marks must stay visible throughout your treatment but they can be washed off once
the course of treatment is over. Sometimes small permanent marks (like tattoos) may
be made on your skin. At the beginning of your treatment, you will be given
instructions on how to look after your skin in the area being treated, as radiotherapy
can make the skin sore.

Treatment sessions

Before each session of radiotherapy, the radiographer will position you carefully on
the couch and make sure that you are comfortable. During your treatment, which only
takes a few minutes, you will be left alone in the room, but you will be able to talk to
the radiographer who will be watching you carefully.

Radiotherapy is not painful, but you do have to lie still for a few minutes while your
treatment is being given. The treatment will not make you radioactive and it is
perfectly safe for you to be with other people, including children, after your treatment.

Positioning the radiotherapy machine

Side effects

Radiotherapy can cause general side effects such as feeling sick and tiredness. The
side effects you get will depend on the area of the body being treated and the length
of your treatment. The clinical oncologist and radiographer will advise you what to
expect.
 26

Hair loss

Hair will only fall out in the area being treated by radiotherapy, so the treatment for
soft tissue sarcomas will not make the hair on your head fall out. The hair that is lost
may grow back after the treatment has ended, but is often lost permanently.

Skin care

During radiotherapy, the skin in the treated area may become darker or red and sore.
Your radiotherapist will give you advice about skin care at the start of your treatment.
Your doctor can prescribe a special cream to soothe sore skin if necessary.

Tiredness and fatigue

As radiotherapy can make you tired, try to get as much rest as you can, especially if
you have to travel a long way for treatment each day.

Feeling sick

If nausea and vomiting occur they can usually be effectively treated with anti-
sickness drugs (called anti-emetics), which your doctor can prescribe. If you don’t
feel like eating, you can replace meals with nutritious, high-calorie drinks which are
available from most chemists and can be prescribed by your GP. Our section on diet
has some helpful hints on how to eat well.

All these side effects should disappear gradually once the course of treatment
is over, but it is important to let your doctor know if they continue.

Long-term side effects

After radiotherapy, some people may develop swelling known as lymphoedema. This
happens because the lymph glands and vessels can become damaged by the
radiotherapy. Lymph fluid (which circulates around the lymphatic system) is unable to
pass along the vessels and builds up, causing swelling.

If lymphoedema develops it can’t be cured, but it can often be treated and managed.
To help prevent lymphoedema you should try to avoid getting any infection or
inflammation in the area that has been treated with radiotherapy. You should try to
avoid cuts or grazes in the area and help to look after your skin by using moisturisers
if it gets dry.

Radiotherapy to a joint, such as the knee or elbow, may cause it to become stiff. To
help prevent stiffness, it is important to keep the joint mobile by using it and doing
regular exercise.

Our section on radiotherapy gives more detail about this treatment and side effects.

Chemotherapy for soft tissue sarcomas

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. As
the drugs are carried in the blood, they can reach cancer cells all over the body.
 27

Chemotherapy may sometimes be given before surgery, to shrink the tumour and
make it easier to remove. This is known as neo-adjuvant treatment.

Chemotherapy may also sometimes be used after surgery, to try to destroy any
cancer cells that were too small to be removed, or which may have spread to other
parts of the body. This is known as adjuvant treatment.

Certain types of soft tissue sarcomas are always treated with chemotherapy. For
other types it is very rarely used. Your doctor can tell you whether chemotherapy will
be used to treat your type of soft tissue sarcoma.

Giving chemotherapy
Side effects

Giving chemotherapy

Chemotherapy drugs are usually given by injection into a vein (intravenously) but
sometimes can be given as tablets. The most commonly used chemotherapy drugs
for soft tissue sarcomas are doxorubicin and ifosfamide.

Chemotherapy is given as a session (or cycle) of treatment, which usually lasts a few
days. This is followed by a rest period of a few weeks to allow your body to recover
from any side effects of the treatment.

The number of cycles you have will depend on the type of sarcoma you have and
how well it is responding to the drugs. If doxorubicin is given on its own you will be
given it as an outpatient. You will usually be given any other types of chemotherapy
as an inpatient, which means spending a few days in hospital.

Side effects

Chemotherapy can cause unpleasant side effects, but it affects everyone differently
and any side effects that do occur can often be well controlled with medicines.

Lowered resistance to infection

Chemotherapy can reduce the production of white blood cells by the bone marrow,
making you more likely to get an infection. This effect can begin about seven days
after treatment has been given and your resistance to infection usually reaches its
lowest point 10–14 days after chemotherapy. Your blood cells will then increase
steadily and will usually have returned to normal before your next course of
chemotherapy is due.

Contact your doctor or the hospital straight away if:

your temperature goes above 38ºC (100.5ºF)

you suddenly feel unwell (even with a normal temperature).

You will have a blood test before each cycle of chemotherapy, to make sure that your
cells have recovered. If necessary, you may be given antibiotics to treat any
infection. Occasionally it may be necessary to delay your treatment if your blood
count is still low. We can send you information about how to avoid an infection if you
have reduced immunity.
 28

Bruising or bleeding

The chemotherapy can also reduce the production of platelets, which help the blood
to clot. Let your doctor know if you have any unexplained bruising or bleeding, such
as nosebleeds, blood spots or rashes on the skin, and bleeding gums.

Anaemia (low number of red blood cells)

While having chemotherapy, you may become anaemic. This may make you feel
tired and breathless.

Feeling sick

Some of the drugs used to treat soft tissue sarcomas may make people feel sick
(nauseous) and vomit. There are now very effective anti-sickness drugs (anti-
emetics) to prevent or greatly reduce nausea and vomiting. Your doctor can
prescribe these for you. Let your doctor know if you still feel sick, as they can
prescribe other types of anti-sickness drugs for you.

Sore mouth

Some chemotherapy drugs can make your mouth sore and cause small ulcers.
Regular mouthwashes are important and your nurse will show you how to do these
properly.

Taste changes

You may notice that your food tastes different. Your taste will usually go back to
normal after the treatment finishes. If you don’t feel like eating during treatment, you
could try replacing some meals with nutritious drinks or a soft diet – our section on
eating well has some useful tips on coping with eating problems.

Hair loss

Unfortunately, hair loss is a common side effect of some chemotherapy drugs. You
can ask your doctor whether the drugs you are taking are likely to make your hair fall
out.

It may be possible to reduce the amount of hair that you lose by using scalp cooling.
Cooling the scalp during chemotherapy means that fewer chemotherapy drugs reach
the hair follicles, and so the hair is less likely to fall out. We can send you information
about scalp cooling.

People who lose their hair often cover up by wearing wigs, hats or scarves. Your
nurses can arrange for you to see a wig-fitter to help you choose a style and colour
that suits you. If your hair falls out, it will grow back over a period of 3–6 months once
your treatment has finished.

Tiredness

You may feel tired and have a general feeling of weakness. It is important to allow
yourself plenty of time to rest.
 29

Although they may be hard to bear at the time, these side effects will disappear
over a few months once your treatment is over.

Fertility

Your ability to become pregnant or father a child may be affected by some of the
chemotherapy drugs used to treat sarcomas. It is important to discuss fertility with
your nurse or doctor before starting treatment as it may be possible for men to store
sperm and women to store eggs or embryos for use in the future.

Some women may find that the chemotherapy treatment causes an early
menopause, and they may have the signs of the menopause, such as hot flushes
and sweats. In many cases, HRT (hormone replacement therapy) can be given to
replace the hormones that are no longer being produced. Women with
gynaecological sarcomas may not be able to have HRT because the cancer may be
sensitive to hormones.

You may find it helpful to talk all this through with your doctor or one of the support
organisations.

Contraception

It is not advisable to become pregnant or father a child while having any of the
chemotherapy drugs used to treat sarcomas, as they may harm the developing
foetus. It is important to use effective contraception during your treatment and for up
to a year afterwards. You can discuss this with your doctor or specialist nurse.

Condoms should be used during sex within the first 48 hours after chemotherapy, to
protect your partner from any of the drug that may be present in semen or vaginal
fluid. Again, you can discuss this with your doctor.

It is important to remember that chemotherapy affects different people in different

ways. Some people find they are able to lead a fairly normal life during their
treatment, but many become very tired and have to take things much more slowly.
Just do as much as you feel like and try not to overdo it.

Biological therapies for soft tissue sarcomas

Biological therapies are treatments based on substances that are produced naturally
in the body to destroy cancer cells.

Imatinib (Glivec®)
Sunitinib (Sutent®)

Imatinib (Glivec®)

Imatinib is a type of drug known as a tyrosine kinase inhibitor. It is sometimes used to

treat gastrointestinal stromal tumours (GISTs). Imatinib works by blocking (inhibiting)
signals within cancer cells and preventing a series of chemical reactions that make
 30

the cells grow and divide. The chemical it blocks is called tyrosine kinase. The effects
of imatinib are very specific to GISTs. It is taken once a day as tablets.

Imatinib can cause side effects including feeling sick (nausea), vomiting, diarrhoea, a
skin rash and puffiness, especially around the eyes. Some of the side effects can be
reduced with other medicines.

Imatinib can sometimes control GISTs for several years.

Sunitinib (Sutent®)

Sunitinib is another tyrosine kinase inhibitor. It is sometimes used to treat GISTs that
don’t respond to imatinib, or if the side effects of imatinib are too much. As well as
blocking signals within the cancer cells and preventing a series of chemical reactions
that make the cell grow and divide, it helps to prevent the tumour developing new
blood vessels. Without blood vessels the tumour can’t get the nutrients it needs to
survive.

Sunitinib has recently been licensed as a treatment for GISTs, but it may not be
widely available. Your doctor can advise you if sunitinib is suitable for you.

Side effects of sunitinib include tiredness, diarrhoea, sore hands and feet, a sore
mouth, taste changes and a loss of appetite.

Follow-up after treatment for soft tissue sarcomas

While most people recover well after surgery for soft tissue sarcomas, and are able
to move around quite soon, some people take longer and need extra help. Before
you go home, the medical staff will talk to you about your situation. If you live alone
or have several stairs to climb, you may need some help at home.

If you have any worries about going home, make sure you discuss them with the
medical staff in advance so that help can be organised. Children and teenagers often
worry about falling behind at school. Home tutoring can often be arranged with the
local education authority. The social worker on your ward will be able to organise this
for you.

After your treatment has ended your doctor will want you to have regular check-ups
and x-rays, in particular chest x-rays. These will often continue for several years. If
you have any problems or notice any new symptoms in between these times (for
example, a lump or swelling at the area of your operation), let your doctor know as
soon as possible.

The gaps between your appointments will get longer as the years go on. This is
because the risk of the cancer coming back gets steadily lower over time.

What if the cancer comes back?

Sometimes, the sarcoma may come back or spread to other parts of the body
including, most often, the lungs.
 31

If tests show that you have a small amount of cancer in your lungs, it may be
possible for you to have an operation to remove the part of the lung that is affected. If
this operation is necessary in your case, your doctor will discuss it fully with you.

If the cancer comes back elsewhere, your doctor will talk to you about the different
treatment options available in your situation. The most common treatment for people
in this situation is chemotherapy.

Living with an amputation

Developments in surgery mean that many people with soft tissue sarcomas are now
able to have limb-sparing surgery instead of an amputation. Unfortunately, some
people will still need to have a limb amputated to treat their cancer. Amputation is, of
course, very distressing and can take a long time to come to terms with.

Feelings and emotions

Losing an arm or a leg can feel like a bereavement. You will need time to grieve for
your loss and to start to cope with the emotional and practical difficulties this type of
surgery can bring. Our section on the emotional effects of cancer discusses the
emotions and feelings that a diagnosis of cancer can cause, and also the people and
organisations available to help you to cope with them.

Body image

Even if you thought you had a good idea of what to expect before surgery, you may
still feel shocked and distressed after the operation, when the full realisation of
having lost an arm or leg hits you. You will be used to what your body looks like and
it can be very difficult to come to terms with a major change such as an amputation.

The sense of looking different from other people can seriously affect your self-
confidence and make you afraid of being rejected – both socially and sexually. At
times, you may even wish you had never agreed to the operation.

It may not be easy to let other people see you after your amputation. As you and the
people close to you become more used to the way you look, you will become more
confident about dealing with the reactions of people you don’t know so well.

Some people find it helpful to get out and about as soon as possible after the
operation. However, it is important to take the time you need to get used to your
amputation and to do things in your own time. You may want to take someone with
you at first to give you emotional support. You may find that other people do not even
notice your amputation, especially if you are wearing an artificial limb.

Help is available

You will need time and help to come to terms with your emotions, which are likely to
be very strong. The staff on the ward will know this and will help you all they can.
Often, there are counsellors or psychologists within the hospital, and the ward staff
can arrange for you to see them.
 32

Many people find it helpful to discuss their feelings in depth with a close friend or
someone who is removed from their situation, like a counsellor. Support groups can
give practical help and emotional advice, and can help stop you feeling as if you have
to cope alone.

You may find it helpful to talk to someone who has had an amputation, either before
or after your operation. The doctor or nurses on your ward may be able to arrange
this for you.

Family and friends

Your partner, family and friends may also find it hard to come to terms with their
feelings about your amputation. You may be anxious about what they will say or
think, and whether you will be able to cope with their reactions.

This worry can feel very real, but most people usually find that their families and
friends do not reject them, and want to do as much as possible to support them. It
can help to be open about any fear of rejection.

Our section on talking about your cancer can help you find ways of talking to family
and friends.

Sexuality

You may find that you feel unattractive and embarrassed about your body, and worry
that no one will find you sexually attractive again. If you have a partner, you may be
concerned that they will not find you attractive any more. Meeting new partners may
seem particularly daunting. Our section on sexuality and cancer discusses these
issues.

Research - clinical trials for soft tissue sarcomas

Cancer research trials are carried out to try to find new and better treatments for
cancer. Trials that are carried out on patients are known as clinical trials.

Clinical trials may be carried out to:

test new treatments, such as new chemotherapy drugs, gene therapy or

cancer vaccines
look at new combinations of existing treatments, or change the way they are
given, to make them more effective or to reduce side effects
compare the effectiveness of drugs used to control symptoms
find out how cancer treatments work
see which treatments are the most cost-effective.

Trials are the only reliable way to find out if a different operation, type of
chemotherapy, radiotherapy, or other treatment is better than what is already
available.
 33

Taking part in a trial

You may be asked to take part in a treatment research trial. There can be many
benefits in doing this. Trials help to improve knowledge about sarcomas and develop
new treatments. You will be carefully monitored during and after the study. Usually,
several hospitals around the country take part in these trials. It is important to bear in
mind that some treatments that look promising at first are often later found not to be
as good as existing treatments, or to have side effects that outweigh the benefits.

If you decide not to take part in a trial your decision will be respected and you do not
have to give a reason. There will be no change in the way that you are treated by the
hospital staff and you will be offered the best standard treatment for your situation.

Blood and tumour samples

Many blood samples and bone marrow or tumour biopsies may be taken to help
make the right diagnosis. You may be asked for your permission to use some of your
samples for research into cancer. If you are taking part in a trial you may also be
asked to give other samples which may be frozen and stored for future use, when
new research techniques become available. These samples will have your name
removed from them (anonymised) so you can’t be identified.

The research may be carried out at the hospital where you are treated, or it may be
at another hospital. This type of research takes a long time, so you are unlikely to
hear the results. The samples will, however, be used to increase knowledge about
the causes of cancer and its treatment. This research will, hopefully, improve the
outlook for future patients.

Current research

If you have a sarcoma that has spread to another part of the body or come back
(recurred) after treatment, you may be asked to take part in a trial using the
chemotherapy drugs doxorubicin and ifosfamide. This is a randomised trial and half
of the people will be treated with doxorubicin only. The other half will receive a
combination of the two drugs.

If you have a leiomyosarcoma that can’t be removed with surgery, or has spread to
another part of the body, you may be asked to take part in a chemotherapy trial. The
trial involves treatment with the chemotherapy drugs gemcitabine (Gemzar®) and
docetaxel (Taxotere®).

A biological therapy is being tested as a treatment for people with two rare soft tissue
sarcomas that affect the skin. The treatment, called imatinib (Glivec®) is a tyrosine
kinase inhibitor and is being given to people with a dermato fibrosarcoma
protuberans (DFSP) or a giant cell fibroblastoma (GCF).
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JASCAP resources
Talking about your cancer
Practical advice and guidance for cancer patients to help them communicate with
family, friends, carers and health professionals about emotional and practical issues
arising from a diagnosis of cancer and cancer treatment.

Talking to children about cancer

Practical advice and guidance to help parents with cancer talk to their children about
their cancer.

Talking to someone with cancer

Practical advice and guidance for friends, carers and family members to help them
talk to their friend or relative with cancer, and provide emotional and practical
support.

Note: JASCAP has booklets on each of the above subjects.

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Questions you might like to ask your doctor or surgeon

You can fill this in before you see the doctor or surgeon, and then use it to remind
yourself of the questions you want to ask, and the answers you receive.

1. _______________________________________

Answer _______________________________________

_____________________________________________

2. _______________________________________

Answer _______________________________________

_____________________________________________

3. _______________________________________

Answer _______________________________________

_____________________________________________

4. _______________________________________

Answer _______________________________________

_____________________________________________

5. _______________________________________

Answer _______________________________________

_____________________________________________
 36

JASCAP : We need your help

We hope that you found this booklet useful.

To help other patients and their families we need and intend to extend our Patient
Information Services in many ways.

Our Trust depends on voluntary donations. Please send your donation by Cheque or
D/D payable in Mumbai in favour of “JASCAP”.

Note for Reader

This JASCAP booklet is not designed to provide medical advice or professional

services and is intended to be for educational use only. The information provided
through JASCAP is not a substitute for professional care and should not be used for
diagnosing or treating a health problem or a disease. If you have, or suspect you may
have, a health problem you should consult your doctor.
 37

JASCAP

JEET ASSOCIATION FOR SUPPORT TO CANCER PATIENTS,

C/O ABHAY BHAGAT & CO., OFFICE NO.4, “SHILPA”,
7TH. ROAD, PRABHAT COLONY,
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MUMBAI - 400 055.
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FAX: 91-22-2618 6162,
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NEAR JUDGES’ BUNGALOWS,
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455, I CROSS,
HAL III STAGE,
BANGALORE – 560 075
PHONE : 91-80-2528 0309 .
e-mail : supriyakgopi@yahoo.co.in

HYDERABAD: MS. SUCHITA DINAKER & DR. M. DINAKER, M.D.,

FLAT NO. G4, 1ST. FLOOR, “STERLING ELEGANZA”,

STREET NO.5, NEHRUNAGAR,
SECUNDERABAD – 500 026.
PHONE : 91-40-2780 7295.
e-mail : suchitadinaker@yahoo.co.in