ROYAL MARSDEN HOSPITAL

CHILDREN'S DEPARTMENT

EWING'S SARCOMA

EWING'S SARCOMA UPDATE (MARCH 1994)

The treatment protocol which we now use in most cases is called EICESS, (European

Intergroup Co-operative Ewing's Sarcoma Study).

This treatment takes nearly one year; much of that time will be spent as an in patient on the

children's ward and occasionally at your local hospital.

Treatment

Please refer to the section starting on page 5.

In EICESS the treatment blocks contain different combinations of drugs and so have different

names to those in ET2 (the previous study).

The names of the treatment blocks in EICESS are:

VAIA This contains vincristine, actinomycin D, ifosfamide and adriamycin (also called doxorubicin).

EVAIA This contains vincristine, actinomycin D, ifosfamide, adriamycin (also called doxorubicin and etoposide.

VACA This contains vincristine, actinomycin D, cyclophosphamide and adriamycin (also called doxorubicin).

In addition to the drugs listed in the leaflet one other may be used.

Etoposide (VP16)

This is either given as capsules or as an intravenous infusion over 1 hour or 4 hours. The side effects of this drug are:

Common - Nausea and vomiting

- Hair loss

- Bone marrow suppression

- Occasional Unpleasant reaction if given too fast

EWING'S SARCOMA

Your child has a tumour called Ewing's sarcoma, this is a type of cancer which can develop anywhere in the body, but usually starts in a bone. The commonest bone to be involved is the pelvis, upper arm or thigh. Occasionally it starts in the soft tissue near a bone. With the correct treatment nearly 60% of children with Ewing's sarcoma are cured. The treatment protocol which we use is called E.T.2 (Ewing's Tumour, second United Kingdom study). The treatment takes one year; much of the time will be spent as an in-patient on the children's ward and occasionally at your local hospital.

INVESTIGATIONS

In order to provide your child with the most appropriate treatment we need to perform several tests (investigations) which look at the tumour itself, parts of the body it might spread (metastasise) to and parts of the body which might be affected by the treatment. We look particularly carefully at the chest (with chest x-ray and CT scan) because this is a very common place for Ewing's sarcoma to spread to.

These tests are done before treatment starts and at stages throughout the year.

The investigations your child will need are:

Blood tests

Your child will need frequent blood tests both whilst having chemotherapy and between courses. If your child does not have a Hickman line, we will use local anaesthetic cream to numb the skin before doing a blood test. (Hickman line is explained later).

X-rays

Of the chest and area affected by the tumour.

Biopsy

This is a limited operation, usually under general anaesthetic, to remove a small piece of tissue from which a diagnosis can be made.

Bone marrow aspirate and trephine

As some cancer affects the bone marrow this is examined before treatment starts and at stages during treatment. A needle is put into the bone (usually the hip) and some marrow sucked out (aspirate). Sometimes a tiny piece of bone is also taken (a trephine). These samples are studied in the laboratory. An anaesthetic is always used, most children will have a general anaesthetic, although some older children choose a local anaesthetic. In some cases bone marrow is taken from more than one place.

Afterwards there is a small pinprick covered by a plaster. Your child may have a small bruise or be a bit stiff for a few days.

Computerised axial tomography (CAT or CT scan)

Computerised images are taken using x-rays. Your child has to lie on a couch which moves into a large ring containing the sensors. The scan does not hurt, but it is important to keep still. Small children will require a general anaesthetic or sedation. Sometimes a harmless dye (contrast) is ~ised, this may be injected or swallowed depending on the scan. The scan takes abotit half an hour and a parent can usually stay with their child.

Magnetic resonance imaging (MRI or NMR)

The way magnetic waves are altered as they pass through different cells and tissues in the body is used to make a computerised image of the part of the body scanned. Your child will lie on a couch which moves into a large ring (the magnet). It does not hurt, there is no radiation and there are no side effects but it is very noisy. The scan can take up to an hour and it is important to keep still, so small children may need to be sedated. Parents can stay in the room with their child.

Bone scan

This looks for particular problems in bones, such as a tumour or a metastases from a tumour. A tiny amount of radioactive dye is injected on the day of the scan. This is quite harmless as the amount of radioactivity is very small.

A few hours later the scan is done. Your child lies on a couch with the camera above him. This does not hurt and takes about half an hour.

Urine

We often collect samples of urine for a number of reasons 1) to look for infection, 2) to assess response to treatment and 3) to detect complications of treatment. In addition, your child's fluid balance (how much fluid goes in and how much comes out) is important to monitor during chemotherapy. You will be required to keep details on how much urine is passed and chart this on the fltud chart. If there is any difficulty, the nursing staff will assist you with this task.

EDTA (kidney test)

Some of the drugs that your child will receive may have an effect on the kidneys. We will test the kidney function at regular intervals, this will involve an injection of a tiny amount of radioactive substance and a number of blood tests (usually three) over a few hours. Should the chemotherapy we use prove to be damaging the kidneys, the doses will be adjusted for the next chemotherapy courses.

Echocardiogram

This is an examination of the heart performed by ultrasound (i.e. like a pregnancy scan). It is not painful but requires that your child lies still and have some jelly applied to the chest and upper abdomen. This scan will be performed at another hospital between treatments and is to monitor heart function which may be affected by some drugs.

GENERAL INFORMATION

General Anaesthetic

Genera] anaesthetics (GAs) are often used if your child needs a painful procedure, or if it is important that he keeps very still.

Unfortunately there is always a small risk of a serious problem occurring with a general anaesthetic, so there may be occasions when we delay the procedure or use sedation instead. If your child has a heavy chest cold the GA may be postponed for a few days.

All GAs are performed by experienced anaesthetists and your child is monitored carefully until he/she has recovered fully.

Hickman line

This is permanent tube which is inserted into the bloodstream through which blood samples can be taken and drugs and fluids given. It is put in through a small cut in the neck, usually under general anaesthetic. From here the line runs just underneath the skin to come out on the side of the chest. This line avoids the need for repeated needle pricks and allows chemotherapy to be given more safely. You will be taught how to look after it. Despite good care, Hickman lines may become infected, in which case we will usually treat your child with intravenous (IV) antibiotics. If this does not clear the infection, the line will be removed and replaced at a later date if still needed.

Bone Marrow Suppression (myelosuppression or myelotoxicity).

The bone marrow is where the blood cells are produced, this is an ongoing process as blood cells are continually being used up; The number of blood cells can easily be measured (a full blood count). This measures the three main parts of blood, the haemoglobin (Hb), platelets and white blood cells (WBC). The white blood cells help the body to fight infection, platelets help to stop bleeding and bruising, and anaemia occurs if the Hb is low.

Some drugs decrease the production of blood cells by the bone marrow for a variable period of time. During this time the blood count can be very low and your child may need blood transfusions to correct anaemia, platelet transfusions to stop bruising or bleeding, and he will be at increased risk of infections. All blood and platelets in the UK are fully screened for infection and we consider them to be quite safe.

Infection

Your child is at increased risk of infection because the treatment decreases the production of white blood cells (particularly the neutrophils) by the bone marrow. If your child is unwell in any way, or has a temperature of 38^0C or more, please ring us up for advice. He/she may need to come to the hospital and receive intravenous antibiotics. This is a very common problem.

^{Nausea and Vomiting}

^{Some of the chemotherapy drugs used may make your child feel sick or vomit. If he/she is going to receive these drugs we will give him/her "anti-emetics", these are drugs designed to stop nausea and vomiting and are usually very successful.}

^Mucositis

^{Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. All children must perform their mouth care to ensure their mouth stays as clean as possible. If your child's mouth becomes sore he/she may not want to eat or drink, and may need to be admitted to hospital for pain relief and intravenous fluids. These are common problems following some treatments and fully reversible.}

^{Sperm Banking}

^{Although the chemo- or radiotherapy that your soil will receive as part of his treatment will not have any effect on sexual function in later life, it may render him sterile and unable to father children. With this in mind, if your son is of a suitable age, we will attempt to store a sample of semen so that the sperm could be used later.}

^Photographs

^{We may ask you to agree to photographs being taken of your child before and during treatment. These will be used to provide a visual record of the illness, and they will be filed in your child's hospital notes. In addition, you may be asked to allow photographs to be used as teaching aids for nursing or medical staff.}

^TREATMENT

^{The treatment your child will recieve consists of chemotherapy (drugs), surgery (an operation on the tumour) and/or radiotherapy (x-ray treatment).}

^{Chemotherapy is given as an in-patient in blocks of treatment lasting 3 - 6 days. Each block is given three weeks apart. After each treatment block you will be able to go home if your child feels well enough. However, you will be seen frequently in out-patients in the interval between treatments. It is very likely that your child will need to be readmitted with a treatment related problem between courses of chemotherapy (i.e. with infection or sore mouth).}

^{Each treatment block consists of two or three different drugs. The names of the blocks are:}

^{IVAD 3 - This contains lfosfamide, Vincristine, Adriamycin (also called}

^{Doxorubicin).}

^{IVAD 2 - The same drugs as IVAD 3, but less of them.}

^{IVA - This contains Vincristine, Ifosfamide and Actinomycin D.}

^{V or CV - This is Vincristine with either Ifosfamide or Cyclophosphamide.}

^{ABOUT THE DRUGS}

^{Vincristine (Oncovin)}

^{This drug is given as an intravenous injection. The side effects of this drug are}

^{Common - Constipation}

^{- Discomfort on infusion}

^{- Jaw pain, general aches}

^{Rare - Weakness in an arm or leg - this usually recovers.}

^{- "Vincristine burn" - if the drug is given into a vein in the hand it very rarely leaks out and causes a burn.}

^Ifosfamide

^{This is given as a continuous intravenotis infusion over either 3 hours a day for 3 days or continuously for 3 - 5 days.}

^{To prevent bladder problems it is given with large amounts of intravenous fluid and intravenous mesna (a drug to protect the bladder) until 12 hours after the ifosfamide has finished.}

^{The side effects of this drug are}

^Common

^{- Nausea & Vomiting}

^{- Hair Loss}

^{- Mucositis}

^{- Bone Marrow Suppression}

^{- Permanent Sterility}

^{Occasional - Bladder problems - inflammation and bleeding of the bladder lining. This is preventable with mesna.}

^{Rare - Drowsiness}

^{- Altered kidney function (this is monitored and the dose of ifosfamide may need to be altered).}

^{Doxorubicin (Adriamycin)}

^{This is given as an intravenous infusion over 6 hours. The side effects of this drug are}

^{Common Red urine}

^{- Nausea and vomiting}

^{- Bone marrow suppression}

^{- Mucositis}

^{- Hair loss}

^{Rare Heart problems. If your child is going to have a lot of this drug we will perform tests of heart function (echocardiogram)}

^{Actinomycin D}

^{This is given as an intravenous injection. The side effects of this drug are}

^Common

^{- Nausea and vomiting}

^{- Bone marrow suppression}

^{- Mucositis}

^{- Hair loss}

^{Cyclophosphamide}

^{This is given as an intravenous injection. The side effects of this drug are}

^Common

^{-Nausea and vomiting}

^{-Hair loss}

^{-Bone marrow suppression}

^{-Sterility (this may occur in both boys and girls depending on the dose given and it is usually permanent).}

^Occasional

^{- Bladder problems - inflammation and bleeding of the bladder lining. This is preventable with mesna.}

^{TREATMENT PLAN EWING'S SARCOMA.}

^{4 blocks of IVAD 3 - each 3 weeks apart}

^{Surgery or radiotherapy or both, during these chemotherapy is with CV (weekly) or IV (3 weekly).}

^{3 blocks of IVAD 2 - each 3 weeks apart}

^{8 - 10 blocks of IVA - each 3 weeks apart}

^Surgery

^{After four blocks of treatment (i.e. 12 weeks from the start) your child will have more tests to look at the tumour and decide if surgery is needed. You may need to visit Stanmore Orthopaedic Hospital to discuss this, if surgery is going to be done it will be at Stanmore. If surgery is not going to be performed your child will receive radiotherapy at this stage.}

^{An operation is performed to remove the bone and tissue affected by the tumour.}

^{If this is impossible or very difflcult, radiotherapy is used instead. Because the tumours usually occur in the bone it is often necessary to remove one or more bones. At the same time an artificial bone is put in place if necessary so that your child can lead a normal life. The artificial bone (prosthesis) is made specially for your child, and may need to be altered as he grows.}

^Radiotherapy

^{This is treatment which uses x-rays to kill cancer cells. It takes place in a special room in the Radiotherapy Department and lasts a few minutes every day for 2 - 6 weeks. It is given to the area affected by the tumour if surgery is not possible, or if some tumour is left behind after the operation.}

^{It does not hurt, but the skin sometimes gets red and sore. If the growing part of a bone has to be treated, that bone may not be able to grow properly.}

^FOLLOW-UP

^{After the treatment has finished we will continue to watch your child as an out-patient for several years. We will need to do regular chest x-rays and x-rays or CT scans of the bone affected.}

^{From time to time we may arrange other tests (e.g. looking at the heart, sexual development and growth).}

^{It is the policy of this unit to be honest with you at all times. We will fully discuss your child's disease, investigation results and treatment with you. The aim of the course of treatment your child will receive is to cure him of his disease. As there is a small chance that the disease will come back (relapse or recurrence) we will continue to monitor your child in the Out-Patients Department after his treatment has finished.}

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