Paediatric Oncology

Family-centred care is at the heart of the service provided at the Mediclinic Paediatric Oncology unit. When a child or young person is diagnosed with cancer, the whole family is affected. Because parents and families know their children best, we believe they are central to the care-giving process and we value their input and feedback. Parents are closely involved in treatment plans, and their implementation, both in the hospital and at home.

It is vital that our patients receive seamless care throughout their treatment pathway. This also allows families to spend more time together at home and for patients to remain involved in the life they had before cancer, enjoying hobbies and interests, seeing their friends and going to school.

Our paediatric oncology service is run a by a team that is truly dedicated to your child’s wellbeing. All children who join our Mediclinic paediatric oncology family are treated holistically through researched evidence treatments with consideration their emotional, psychological and physical needs. The team is here to support the whole family unit during this challenging time.

Paediatric oncology is a field of medicine which diagnoses and treats cancer in children. Childhood cancer is rare. About one in 500 children will develop cancer by the age of 15, and most instances of cancer in children happen before the age of five. The chances of surviving cancer as a child is much higher than for adults, and more than 82% of children are completely cured.

Chemotherapy is defined as the administration of specific medication, either as an infusion or as tablets, aiming to destroy the cancer cells in the body.

Some types of cancer can be treated mainly with chemotherapy, such as leukaemia and lymphomas. For other types of cancer, chemotherapy is used after an operation and/or radiation therapy. Here chemotherapy should kill any remaining tumour cells in the body.

However, chemotherapy may also be performed before surgery. This aims to reduce the size of the tumour and to improve the surgery’s prospects of success. When the chances of curing the cancer dwindle, chemotherapy may possibly be performed to alleviate patients’ symptoms and thus improve their quality of life. 

There are four main types of leukaemia:

• Acute lymphoblastic leukaemia (ALL)
• Acute myeloid leukaemia (AML)
• Chronic lymphocytic leukaemia (CLL)
• Chronic myeloid leukaemia (CML)

Acute lymphoblastic leukaemia (ALL) is a form of cancer that affects the lymphocyte-producing cells in the bone marrow.

Lymphocytes are white blood cells that produce antibodies; they are vital parts of the body’s immune system. There are two main types of lymphocytes: B and T cells.

In ALL there is an accumulation in the bone marrow of immature lymphocyte precursor cells, called blast cells. Eventually the production of normal blood cells is affected by this, resulting in a reduction in the numbers of red cells, normal white cells and platelets in the blood.

ALL is the only form of leukaemia that is more common in children than adults. It is the single most common form of paediatric cancer, accounting for about one-third of all cases in children. About 85% of cases of childhood leukaemia are ALL and it occurs in about 400 children in the UK each year. ALL occurs mostly between the ages of about two and four years. Males are affected more often than females at all ages.

What causes ALL?

There is no single proven cause of childhood ALL but there are a number of suggested causes, some of which are more controversial than others.

Children exposed to higher levels of ionising radiation before they are born or in early life have been shown to have a high risk of developing childhood leukaemia. One theory is that delayed exposure to a common infection may rarely result in the development of leukaemia.

There is no evidence proving that exposure to high-voltage power lines causes leukaemia.

Symptoms

Many of the symptoms of ALL are due to the fact that the leukaemia cells multiply in the bone marrow and stop the production of healthy blood cells. Common symptoms include:

• Tiredness
  
• Lethargy
• Pallor
• Bruising
• Fever and infection
• Swollen glands
• Limb pains

Tests and investigations

Doctors will need to carry out tests to help decide the best treatment for your child. These tests include:

• Blood tests to show if there are any leukaemia cells in the bloodstream and how the other blood cells are affected
• Chest x-ray to show whether any of the lymph glands in the chest are enlarged
• Bone marrow aspirate to show what type of ALL your child has
• Lumbar puncture to see whether there are any leukaemia cells in the spinal fluid

Treatment for acute lymphoblastic leukaemia

The aim of treatment for acute lymphoblastic leukaemia (ALL) in children is to achieve a state called remission where almost all leukaemia cells have been killed and, by further treatment, to completely eradicate the disease and achieve a cure.

ALL needs intensive treatment with chemotherapy initially followed by a prolonged maintenance phase of less intensive chemotherapy. The exact details of treatment will depend upon factors such as age, the number of leukaemic blasts in the blood at diagnosis, the response to treatment and the genetic features of the leukaemic cells. Following the initial period, most of the treatment is administered as an outpatient. Boys usually receive approximately three years of treatment and girls two years.

These are the different phases of treatment:

Induction

This usually consists of intermittent chemotherapy given over approximately four weeks. The aim is to destroy as many leukaemia cells as possible.

Response to the induction will be checked by examining the bone marrow after one to two weeks of treatment and at the end of induction. The aim is for there to be a significant reduction in the number of leukaemic blasts after one to two weeks and no evidence of leukaemia in your child’s bone marrow after induction is completed (remission).

Post-remission therapy

Further chemotherapy is given to destroy any leukaemia cells that may be left and prevent the leukaemia coming back. The number of courses may vary if your child takes part in a clinical trial. This is explained in more detail in the specific trial information sheet.

Central nervous system (CNS) directed therapy

ALL sometimes develops around the brain and spinal cord. This can be prevented by injecting anti-cancer drugs directly into the spinal fluid (intrathecal chemotherapy) during a lumbar puncture. This is administered more frequently in the induction and post-remission phases, and then usually every three months during maintenance therapy.

Continuation or maintenance therapy

This involves giving low-dose daily oral chemotherapy and monthly injections of chemotherapy. The dose of chemotherapy is adjusted according to a weekly blood count. Usually the children are reasonably well during this phase of treatment and the visits to hospital should be less frequent.

Bone marrow transplant

This is reserved for a few children who don’t do so well with standard chemotherapy or if the leukaemia comes back. The chromosome studies will help your doctors decide whether your child needs a bone marrow transplant. About 2–3% of children require a bone marrow transplant.

Effects of treatment

Many children with acute lymphoblastic leukaemia (ALL) are now cured with current treatment. However, the prognosis depends on how well your child responds to treatment and some of the features of the leukaemic cells such as the chromosomes.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low levels of red blood cells (anaemia), white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. However, anti-sickness drugs can be given at the same time to stop nausea and vomiting.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. Your doctor will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Long-term effects

A small number of children may develop long-term side effects related to the treatment. These include impaired heart function and a small increased risk of a second cancer in later life.

A few children are treated with cranial radiotherapy – this may result in problems with growth and puberty and reduced educational achievement.

Once treatment has finished your child will continue to be seen in the outpatient department where doctors will help treat any long-term effects.

AML is when too many immature myeloid blood cells (blasts) are produced in the bone marrow. These are abnormal cells that do not mature into healthy white blood cells.

There are various types of AML depending on which type of cell is affected and how differentiated (well developed) the cell is. They are all treated in the same way except for one type called acute promyelocytic leukaemia (APL).

Who gets acute myeloid leukaemia?

AML is diagnosed in about 100 children in the UK each year. It can occur at any age and boys and girls are equally affected.

Some children with a genetic disorder, such as Down syndrome or Li-Fraumeni syndrome, have an increased risk of developing leukaemia. Rare conditions, such as aplastic anaemia or myelodysplasia, are also associated with an increased risk of leukaemia. There is no evidence to suggest a link between nuclear power plants or high voltage lines.

Signs and symptoms

Many of the symptoms are related to the fact that the leukaemia cells multiply in the bone marrow and stop the production of healthy blood cells. Common symptoms include:

• Tiredness
• Lethargy
• Pallor
• Bruising
• Fever and infection
• Swollen glands
• Limb pains

Tests / investigations

We will need to carry out some tests to find out as much as possible about the type of leukaemia. This will help us to decide on the best treatment for your child. These tests include:

Blood tests – this will tell us if there are any leukaemia cells in the bloodstream and how the other blood cells are affected.

Chest x-ray – this will tell us whether any of the lymph glands in the chest are enlarged.

Bone marrow aspirate and trephine – these tests tell us exactly what type of AML your child has. We can look at features of the leukaemia cells, such as chromosome changes, which may help us to decide on the best treatment.

Lumbar puncture – this is to see whether there are any leukaemia cells in the spinal fluid. Your child will also need some anti-cancer drugs injected directly into the spinal fluid as part of the treatment.

Treatment of acute myeloid leukaemia

Acute myeloid leukaemia (AML) needs intensive treatment with chemotherapy and in some cases a bone marrow transplant. These are the different phases of treatment:

Induction

This consists of two courses of chemotherapy given about four weeks apart. The aim is to destroy as many leukaemia cells as possible. Response to the induction will be checked by examining the bone marrow after each course. The aim is for there to be no evidence of leukaemia in your child’s bone marrow after induction is completed (remission).

Post-remission therapy

Further chemotherapy is given to destroy any leukaemia cells that may be left and prevent the leukaemia coming back.

Bone marrow transplant

This is reserved for those children who don’t do so well with standard chemotherapy or if the leukaemia comes back. The chromosomes and response after the first course of induction chemotherapy help your medical team to decide whether your child needs a bone marrow transplant. About 20% of children will need a transplant as part of their first lot of treatment.

Central nervous system (CNS)

AML may sometimes develop around the brain and spinal cord. This can be prevented by injecting anti-cancer drugs directly into the spinal fluid (intrathecal chemotherapy) during a lumbar puncture.

The treatment being tested may be aimed at:

• Improving the number of people cured (for example: trying new types of surgery or chemotherapy)
  
• Improving chances of survival
• Relieving the symptoms of the cancer
• Relieving the side effects of treatment
• Improving the quality of life or sense of well-being for people with cancer
• Clinical trials may also involve research aimed at understanding more about the tumour’s biology. You may be asked to allow us to do research on the tumour sample removed at surgery, or on blood samples

Well-run clinical trials have led to a significant improvement in the treatment of children with cancer. If you are asked to consider entering your child into a clinical trial you will be given specific information about the trial before deciding whether to take part.

Effects of treatment

Many children with acute myeloid leukaemia (AML) are now cured with current treatment. However, the prognosis depends on how well your child responds to treatment and some of the features of the leukaemic cells such as the chromosomes.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children may develop long-term side effects related to the treatment. These include reduced fertility, impaired heart function and a small increased risk of a second cancer in later life.

Once treatment has finished we will continue to see your child in the outpatient department. There are doctors available who can help us to treat any long-term effects.

Who gets Hodgkin lymphoma?

About 60 children develop Hodgkin lymphoma in the UK each year. It is rare in children under five years and is more common in teenagers and young adults. Boys and girls are equally affected. The exact cause of Hodgkin lymphoma is unknown. We see it more often in children with immune problems.

Signs and symptoms

The most common symptom is painless swelling of one gland or a group of glands, usually in the neck. Other glands may be enlarged, such as in the armpit or groin.

Other symptoms include:

• Fever
• Night sweats
• Weight loss
• Tiredness or lethargy
• Itching
• Cough or breathlessness

Tests / investigations

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. These tests will include:

Blood tests – these will tell us how well the kidneys are working as well as some other information about general health.

Chest x-ray – this will tell us whether the glands in the chest are enlarged.

Ultrasound scan – this will tell us whether the liver and spleen are enlarged.

CT scan – a scan of your child’s neck, chest and abdomen will be carried out. This will give us more detailed information about which glands are enlarged and if the liver, spleen or lungs are affected.

Biopsy – this is a small operation, usually carried out under general anaesthetic, to remove either part, or all, of a swollen lymph gland.

Staging

Staging refers to the size and position of the lymphoma and whether it has spread. Knowing the particular type and stage of the cancer helps the doctors decide of the most appropriate treatment. The following describes the staging system we use for Hodgkin lymphoma:

Stage I: One group of lymph glands is affected and the lymphoma is only on one side of the diaphragm (the breathing muscle that separates the lungs from the abdomen).

Stage II: Two or more groups of lymph glands are affected and the lymphoma is only on one side of the diaphragm.

Stage III: Lymph nodes are affected above and below the diaphragm. The spleen may also be affected.

Stage IV: The lymphoma has spread beyond the lymph glands, for example to the lungs, liver or bone marrow.

The stage is classified as A if your child does not have fever, night sweats or weight loss, or B if these symptoms are present.

Treatment of Hodgkin lymphoma

Treatment for Hodgkin lymphoma involves chemotherapy, radiotherapy or a combination of both. This depends on the type, stage and response to treatment.

Chemotherapy

A combination of chemotherapy (anti-cancer) drugs is used to destroy the cancer cells. The treatment is normally given every few weeks. The exact combination and number of courses depends on the type and stage of Hodgkin lymphoma.

If your child takes part in a ‘clinical trial’, the treatment is explained in more detail in the specific trial information sheet. An outline of the treatment will be provided in the form of a ‘road map’.

Radiotherapy

Your child may have radiotherapy if the lymphoma is confined to one area or if there is some tumour left after chemotherapy. See the Children’s Unit booklet Radiotherapy treatment for more details about this type of treatment.

The treatment being tested may be aimed at:

• Improving the number of people cured (for example, trying new types of surgery or chemotherapy)
• Improving chances of survival
• Relieving symptoms of the cancer
• Telieving the side effects of treatment
• Improving the quality of life or sense of wellbeing for people with cancer

Clinical trials may also involve research aimed at understanding more about the tumour’s biology. You may be asked to allow us to do research on the tumour sample removed at surgery, or on blood samples.

Well-run clinical trials have led to a significant improvement in the treatment of children with cancer. You can find more general information on clinical trials in the separate information sheet. If you are asked to consider entering your child into a clinical trial you will be given specific information about the trial before deciding whether to take part.

Effects of treatment

The prognosis is good and most children with Hodgkin lymphoma are cured.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These include growth problems, impaired heart and lung function and a small risk of developing a second cancer later in life.

Some children, particularly boys, may have impaired fertility after the treatment. Teenage boys can choose to store sperm for use in later years.

Who gets non-Hodgkin lymphoma?

 It can develop at any age and is slightly more common in boys. It is more common in children with weak immune systems.

Signs and symptoms

The first sign is often painless swelling of a group of lymph glands. Other symptoms include:

• Cough
  
• Breathlessness
• Swelling of the face
• Abdominal (tummy) swelling
• Tiredness or lethargy
• Weight loss
• Poor appetite
• Fever

Tests and investigations

Your doctors will need to carry out some tests to find out as much as possible about the type, position and size of the tumour.

These tests will include:

Blood tests to show how well the kidneys are working as well as information about general health

Chest X-ray to show whether the glands in the chest are enlarged

ultrasound scan to show whether the liver and spleen are enlarged

CT scan to give more detailed information about which glands are enlarged and if the liver, spleen or lungs are affected

biopsy, a small operation, usually carried out under general anaesthetic, to remove either part or all of a swollen lymph gland

Bone marrow aspirate and trephine to show whether the lymphoma has spread to the bone marrow

Lumbar puncture to show whether there are any lymphoma cells in the spinal fluid.

Staging

Staging refers to the size and position of the lymphoma and whether it has spread. Knowing the particular type and stage of the cancer helps the doctors decide on the most appropriate treatment. The following describes the staging system used for NHL:

Stage I: one group of lymph glands is affected or there is a single tumour outside of the lymph glands (extra nodal)

Stage II: two or more groups of lymph glands are affected or there are two extra nodal tumours, but only on one side of the diaphragm

Stage III: lymphoma is present on both sides of the diaphragm, either in two or more groups of lymph glands or two single extra nodal tumours

Stage IV: the lymphoma has spread beyond the lymph glands to other organs such as the bone marrow or nervous system.

Treatment of non-Hodgkin lymphoma

Treatment for non-Hodgkin lymphoma (NHL) in children mainly involves chemotherapy. The exact plan depends on the type, stage and response to treatment.

Chemotherapy

A combination of chemotherapy drugs is used to destroy the cancer cells. The treatment may last a few months or years. The exact combination and length of treatment depends on the type and stage of lymphoma. Chemotherapy may be given by injections or a drip into a vein (intravenous infusion).

NHL may sometimes affect the central nervous system. To stop this happening chemotherapy is injected into the spinal fluid during a lumbar puncture. This treatment is known as intrathecal chemotherapy and is given at regular time intervals during your child’s treatment.

High-dose chemotherapy with stem cell transplant is sometimes used if the lymphoma comes back after initial treatment.

Radiotherapy

Radiotherapy is rarely used to treat NHL. If your child needs radiotherapy your doctor will explain why. More information is available in the Children’s Unit booklet called Radiotherapy treatment.

Surgery

Occasionally, enlarged glands in the abdomen may cause a blockage of the bowel, which may need an operation to relieve it.

Effects of treatment

The prognosis for non-Hodgkin lymphoma is good and the majority of children are cured.

Long-term effects

A small number of children develop side effects many years later because of the treatment they have received. These include growth problems, impaired heart function and a small risk of developing a second cancer later in life.

Some children, particularly boys, may have reduced fertility after treatment. Teenage boys can choose to store sperm for use in later years.

Usually, this tumour occurs in only one kidney (unilateral), although occasionally there may be tumours in both kidneys (bilateral). Sometimes the tumour can spread (metastasise) to other parts of the body, such as the lungs or liver.

Approximately one in ten thousand children get Wilms’ tumour.  It most often affects children below the age of five and is slightly more common in girls than boys.

In most cases there is no known cause for Wilms’ tumour, although a small number of children have a genetic tendency. This is usually obvious, either because other members of the family have had Wilms’ tumour or because the child has certain growth or development problems that have been present from birth. However, for the majority of children with Wilms’ tumour, there is no genetic tendency, nor is there any increased risk in brothers or sisters.

Signs and symptoms

The most common symptom of Wilms’ tumour is swelling of the abdomen (tummy). This is usually painless. Other symptoms may include:

• Nausea and vomiting
  
• Poor appetite
• Weight loss
• Abdominal pain
• Fever
• Red urine due to blood in the urine
• High blood pressure

Tests and investigations

Your doctors will need to carry out some tests to find out as much as possible about the type, position and size of the tumour.

The tests will include:

Urine and blood samples to show how well the kidneys are working as well as some other information about general health

Chest X-ray to show if the tumour has spread to the lungs

Ultrasound scan to show the position and size of the tumour

CT scan to give more detailed information about the tumour and show if it has spread

Biopsy, a small operation, usually carried out under general anaesthetic, in which a surgeon removes a small piece of tissue from the kidney to help make a diagnosis.

Staging

The tumour stage is a measure of how far the tumour has spread beyond the kidney. This can only be measured accurately after surgery to remove the affected kidney. Knowing the particular type and stage of the cancer helps the doctors decide on the most appropriate treatment.

The following describes the staging system used for Wilms’ tumours:

Stage I: the tumour is confined to the kidney and is completely removed at the time of surgery

Stage II: the tumour has broken through the kidney lining but is still completely removed at the time of surgery

Stage III: some of the tumour is left behind at the time of surgery, either because the tumour breaks or because complete surgical removal is not possible

Stage IV: the tumour has spread to other parts of the body, usually the lungs or lymph nodes (glands)

Stage V: there is tumour in both kidneys (bilateral disease).

Treatment of Wilms’ tumour

Treatment for Wilms’ tumour usually includes surgery, chemotherapy or radiotherapy, or a combination of these.

Surgery

All children with Wilms’ tumour will have surgery to remove the affected kidney. If both your child’s kidneys are affected, part of both kidneys may need to be removed. However, we will first give your child chemotherapy to shrink the tumour; this will make the surgery easier when it is carried out later and will reduce the need for radiotherapy.

Radiotherapy

Radiotherapy may be used after surgery to destroy any remaining tumour cells. See the Children’s Unit booklet Radiotherapy treatment for more details on this type of treatment.

Chemotherapy

Chemotherapy is given together with surgery and/or radiotherapy to treat the tumour. The type of chemotherapy will vary in intensity and duration according to the stage of the tumour and its appearance under the microscope (histology).

If your child takes part in a clinical trial, the treatment will be explained in more detail in the specific trial information sheet.

Effects of treatment

The prognosis for Wilms’ tumour is good for all children whatever their tumour stage, and most children are cured.

If the cancer comes back it is usually within the first two years. If this happens further treatment can be given. This may be more chemotherapy and could include surgery, radiotherapy and sometimes high-dose chemotherapy.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

The bone marrow is where blood cells are produced (red blood cells, white blood cells and platelets). Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. White blood cells help the body to fight infection, platelets help to stop bleeding and bruising, and anaemia develops if red blood cells (or haemoglobin) are low. Your child may need blood or platelet transfusions and will be at increased risk of infections.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. Anti-sickness drugs given at the same time are usually very effective at stopping nausea and vomiting.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. Your child will be given painkillers for this.

Hair loss

Temporary hair loss is common.

Long-term effects

When one kidney is removed the other kidney takes over the function of the missing kidney. If tumour has been removed from both kidneys then the remaining kidney tissue continues to function. Very few children have long-term kidney problems.

A small number of children develop side effects many years later because of the treatment they have received. These may include growth problems, reduced fertility, impaired heart and lung function and a small risk of developing a second cancer later in life.

Rhabdomyosarcoma is the most common type of soft-tissue sarcoma in childhood. This is a cancer of primitive muscle cells and can develop anywhere in the body. The most common sites are around the head and neck, bladder, or testes in boys.

It is slightly more common in boys than girls. About two-thirds of rhabdomyosarcomas occur in children under six years of age.

The cause of soft-tissue sarcomas is unknown. There may be genetic factors in some children, for example, those with Li Fraumeni syndrome, have an increased risk of developing rhabdomyosarcoma.

The signs and symptoms will depend on where the tumour develops. The most usual sign is a swelling or lump. Other symptoms may include:

• Blocked nose or discharge
• Swollen or protruding eye
• Tummy (abdomen) swelling, discomfort or difficulty going to the toilet (constipation)
• Blood in the urine or difficulty passing urine

We will need to carry out some tests to find out as much as possible about the type position and size of the tumour. These tests include:

Chest x-ray – to see whether the tumour has spread to the lungs.

Ultrasound scan – this will tell us the position and size of the tumour.

CT scan or MRI scan – a scan of the affected tissue and chest will be carried out. This will give us more information about the tumour and whether it has spread.

Biopsy – this is a small operation, usually under general anaesthetic, to remove some tissue.

Bone marrow aspirate and trephine – these tests will tell us whether the tumour has spread to the bone marrow.

PET/CT scan – this will tell us whether there is spread to other parts of the body

Staging refers to the size of the tumour and whether it has spread. Knowing the stage of the cancer helps the doctors decide on the most appropriate treatment for your child. We use TNM (tumour, node, metastases) staging for soft-tissue tumours with a simplified version as follows:

Stage I: Tumour confined to the organ or tissue of origin with no evidence of lymph node involvement or distant spread (metastases).

Stage II: Tumour involving one or more adjacent organs or tissues, a collection of fluid (malignant effusion) or multiple tumours in the same organ, with no evidence of lymph node involvement or distant metastases.

Stage III: Any of the above with lymph node involvement.

Stage IV: Evidence of distant metastases.

The treatment for soft-tissue sarcoma involves chemotherapy, surgery and radiotherapy. This depends on the site, stage, appearance down the microscope (histology) and response to treatment.

A combination of anti-cancer drugs is used to destroy the cancer cells. The treatment is normally given every one to three weeks. The exact combination and number of courses depends on the response of the tumour and the risk group. The risk group takes into account the stage, type of tumour, where it is and the age of your child. Some children will need high-dose chemotherapy with a stem cell transplant.

An operation may be needed to remove residual tumour following chemotherapy, if the tumour can be removed easily without damaging other tissues.

Radiotherapy may be needed if surgery is not possible, where there is tumour left behind after surgery, in some limb tumours and those which show certain histological features. See the Children’s Unit booklet Radiotherapy treatment for more details on this type of treatment.

About two thirds of children with soft-tissue sarcomas are cured, however for the individual child this depends on the risk group and whether the tumour has spread.

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Hair loss

Temporary hair loss is common.

Specific drugs

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These include growth problems, impaired heart and kidney function, impaired fertility and a small risk of developing a second cancer later in life. Teenage boys have the option to store sperm for use in later years.

About five years after treatment finishes we will transfer your child’s care to our long-term follow-up clinic. Your child will be seen at regular intervals in this clinic, indefinitely, so that we can help with any long-term effects of the treatment.

A neuroblastoma is a malignant (cancerous) tumour, which develops in the nerve cells that run in a chain down the back of the chest and abdomen (the sympathetic chain).

It most often affects children under the age of five and is slightly more common in boys than girls. We do not know what causes neuroblastoma. There is no genetic tendency, nor is there any increased risk in their brothers or sisters.

Neuroblastoma can present in many different ways, but usually it presents as a lump in the tummy (abdomen). Other symptoms include:

• Poor appetite
• Weight loss
• Lethargy
• Constipation
• Difficulty passing urine
• Bruising around the eyes
• Breathlessness
• Leg weakness (this is less common)

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. This will help us to decide on the best treatment for your child. These tests include:

Urine and blood samples – these will tell us how well the kidneys are working as well as some other information about general health. A specific test on the urine will be carried out to measure substances produced by the tumour. These are called ‘tumour markers’. In neuroblastoma two tumour markers are produced: vanillylmandelic acid (VMA) and homovanillic acid (HVA). Levels of these are often elevated at diagnosis.

Chest x-ray – this will tell us if there is tumour in the chest.

Ultrasound scan – this will tell us the position and size of the tumour. We can also use ultrasound scans to monitor the response to treatment.

CT scan – a scan of your child’s abdomen and lungs will be carried out. This will give us more detailed information about the tumour and may tell us if it has spread.

Biopsy – this is a small operation, usually carried out under general anaesthetic. The surgeon will remove a small piece of tissue from the lump, which will help us to make a diagnosis.

MIBG scan – this looks at places to which the tumour might have spread, such as bone, bone marrow and glands (lymph nodes). A mildly radioactive dye is injected which is taken up by neuroblastoma cells, and the scan is carried out the following day. This is quite harmless as the amount of radioactivity is very small. Your child lies on a couch with the camera above him/her and the scan takes about half-an-hour.

Bone marrow aspirate and trephine – as neuroblastoma may affect the bone marrow this is examined before treatment starts and possibly at stages during therapy.

Bone scan – this may tell us if tumour has spread to the bones. A mildly 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 will lie on a couch with the camera moving over him/her. The scan takes about half-an-hour.

Staging is a measure of how far the tumour has spread beyond its original site. The following describes the staging system we use for neuroblastoma:

Stage I: The tumour is localised with or without microscopic residual tumour after surgery.

Stage IIA: The tumour is localised but cannot be completely removed by surgery.

Stage IIB: The tumour is localised but has spread to local lymph nodes.

Stage III: The tumour crosses the midline, with or without local lymph nodes and cannot be removed surgically. There is localised tumour with lymph node involvement on the opposite side. There is midline tumour with lymph nodes on both sides.

Stage IV: The tumour has spread to distant lymph nodes, bone marrow, bone, liver and/or other organs.

Stage IVS: The tumour is localised (stage I, IIA or IIB), with spread limited to liver, skin and/or bone marrow in an infant under one year of age.

Treatment for neuroblastoma depends on the age, size and position of the tumour and whether it has spread. The treatment options include surgery, chemotherapy or radiotherapy, or a combination of these.

Children with stage I and II tumours which can be completely removed will be treated with surgery alone providing the tumour has favourable features on biopsy. Where the tumour cannot be completely removed initially or has spread to other sites then the tumour will be removed after chemotherapy.

Treatment with anticancer drugs is used to destroy the cancer cells. It is usually given by injections and drips into a vein (intravenous infusion).

The type of chemotherapy will vary in intensity and duration according to the stage of the tumour, special features of the tumour (tumour biology) and response. Some children will need high-dose chemotherapy with a stem cell transplant.

Radiotherapy may be used after surgery or high dose chemotherapy to destroy any remaining tumour cells. 

The prognosis for neuroblastoma depends upon the stage of the tumour and its biology.

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These may include growth problems, reduced fertility, hearing problems, impaired heart and kidney function and a small risk of developing a second cancer later in life.

Medulloblastoma/PNET is a malignant (cancer) tumour formed from primitive or poorly developed brain cells

Most of these tumours originate in the lower back part of the brain (called the cerebellum) and are called medulloblastomas.

When they occur in other parts of the brain they are called PNETs (Primitive Neuro Ectodermal Tumours). If a PNET occurs in the area of the brain called the pineal gland, then the PNET is known as a pineoblastoma. All PNETs can sometimes spread (disseminate or metastasise) to the spinal cord through the cerebrospinal fluid. They rarely spread to other organs of the body.

It is the most common malignant brain tumour in children, representing 20% of all childhood brain tumours. They are most common in children between the ages of three and eight, and are slightly more common in boys than girls. They can also be found in adults but are extremely rare.

Like most brain tumours the cause of medulloblastoma or PNET is usually unknown.

The symptoms are usually due to increased pressure in the head (raised intracranial pressure). Symptoms include:

• Nausea and vomiting (most common)
  
• Lethargy and irritability
• Headaches
• Clumsiness
• Difficulty with tasks like handwriting
• Gradual decline in school work
• Changes in personality and behaviour
• Abnormal gait (the way the child walks)

If the tumour spreads to the spinal cord, the signs and symptoms may include:

• Back pain
• Difficulty walking
• Problems with bowel and bladder control

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. This is will help us to decide on the best treatment for your child. These tests include:

CT scan – it is likely that a CT scan of your child’s brain was carried out at your local hospital. Although MRI scans are usually the best way of seeing the tumour and the effects of treatment, sometimes CT scans are also used.

MRI scan – this scan allows us to see the brain and spine in great detail and is used regularly to diagnose and follow the effect treatment is having on your child’s tumour.

Lumbar puncture – this is carried out under general anaesthetic about 14 days after surgery, to look for any malignant cells in the cerebrospinal fluid.

Staging is a measure of how far the tumour has spread beyond its original site. The staging system we use for medulloblastoma / PNET is:

M0: No evidence of metastases.

M1: Tumour cells found in cerebrospinal fluid (lumbar puncture cerebrospinal fluid test).

M2: Tumour spread beyond original site but still in the brain.

M3: Tumour deposits (seeds) in the spine that are easily seen on MRI scan.

M4: Tumour spread to areas outside the central nervous system (brain and spine).

The treatment used for medulloblastoma or PNET depends on where the tumour is, if it has spread and the age of your child. Common treatments include:

Surgery

All children will have an operation to confirm the diagnosis, relieve pressure and try to remove as much of the tumour as possible.

Radiotherapy

Radiotherapy is often used after surgery to destroy any remaining malignant cells. As medulloblastoma / PNET may spread through the cerebrospinal fluid to the spinal cord, radiotherapy is given to the brain and spinal cord.

However, in young children (especially under the age of three years), we may delay, reduce or possibly avoid radiotherapy. This is because of the significant effects of radiotherapy on a younger child’s brain.

Chemotherapy

Chemotherapy is given together with surgery and or radiotherapy to treat the tumour. Treatment with anti-cancer drugs is used to destroy the cancer cells. It is usually given by injections and drips into a vein (intravenous infusion).

The type of chemotherapy will vary according to whether the tumour has spread, if radiotherapy is to be used and the age of the child. Some children may need high-dose chemotherapy with a stem cell transplant.

Significant progress has been made in the management of children with medulloblastoma / PNET over the last ten years.

The majority of children with a localised tumour removed by the surgeon and who also have intensive chemotherapy and radiotherapy in a reduced dose to the brain and spine are cured. Children who have PNET, an incomplete removal of tumour at initial surgery, or evidence of the tumour having spread, have a poorer outlook.

The treatment of infants is difficult. This is because of the need to delay, avoid or reduce radiotherapy to the brain and spine, because of the damaging effects of radiation on the young developing brain.

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Sore mouth (mucositis)

Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

As medulloblastomas are more common in young children and the treatment is given at an important time of the child's development there may be some long-term effects of treatment. These could include growth and hormonal changes, reduced fertility, behavioural changes, possible learning problems and difficulties with coordination. Hearing and visual disturbances can result from both tumour and treatments. There is a small risk of developing a second cancer later in life.

The palliative care team at Mediclinic provide specialist support and advice to patients who have been diagnosed with a life-limiting illness (the patient is considered to be within the last 12 months of life). It is a team of doctors and nurses who are specially trained to provide care for cancer patients  to :

• Improve the quality of life of patients and their families facing the problem of life-threatening illness
• Prevent and relieve suffering
• Identify, assess and treat pain and other problems, physical, psychosocial and spiritual
• Offer a support system to help patients live as actively as possible until death

It is appropriate to receive palliative care  early in the course of illness, in combination with other therapies that are intended to prolong life, such as chemotherapy or radiation therapy.

There are a number of different services that sit under this pathway which work together to provide specialist physical, psychological and spiritual care to patients and carers.

Our team also offer a united approach to providing end of life care, ensuring a seamless and supported journey for patients and their families.

At the Mediclinic Comprehensive Cancer Centre we offer a specialised Cancer Rehabilitation programme that is dedicated to improving the strength, quality of life and sense of wellbeing of patients diagnosed with cancer.  It is specifically designed to help maximise recovery, regain physical and emotional strength, and improve body confidence and control. The programme provides a full range of services designed to care, support and guide patients through every stage of their treatment and recovery.

The cancer rehabilitation programme aims to:

• Optimise physical and functional recovery
• Ease pain and muscular tension
• Help regain strength and mobility
• Increase energy levels and improve breathing
• Strengthen core muscles and improve posture
• Improve physical functioning and maintain independence
• Reclaim body confidence and control
• Reduce treatment related side effects
• Shift focus from illness to wellness

It is suitable after any type of cancer surgery or treatment and accommodates all ages, fitness levels and mobility.

Research shows that remaining physically active is a very important component in your recovery from cancer and the effects of its treatment. Treatments for cancer typically include surgery, chemotherapy, radiation therapy and endocrine therapies. The effects of these treatments commonly cause many patients to develop unwanted side effects which can lead to significant negative impacts on quality of life.  Your cancer rehab physiotherapist can help guide and support you safely back to exercise.

Specialised physiotherapists will assess each patient individually for physical impairments and the effects of their treatment. They then work with each patient to create a rehabilitation program to suit their particular stage in recovery.

The sessions can include a combination of services including: Manual physiotherapy, graduated resistance training, strategies for pain management, individualised clinical pilates, fatigue management and exercise prescription.

Your physiotherapist will help guide, support and encourage you through each phase of your treatment and recovery.

They will teach you what you can do to get stronger again and help you understand why your body is reacting to the treatments in certain ways. They will provide information to help you feel more in control of your body and life again.

An oncology dietitian specialises in helping cancer patients and their caregivers develop a plan for good nutrition. By achieving good overall health, proper nutrition can help reduce the risk of cancer, keep patients healthier during treatment, and combat side effects and illness.

At the Mediclinic Cancer Centre we provide psychosocial care using the holistic approach to cancer care. This means that we focus on you as an individual and aim to address the social, psychological, emotional, spiritual, and functional aspects of your cancer journey. We use a multidisciplinary approach where all the experts who are involved in your care share information and expertise to ensure that you receive the best psychological support available.

We have a psychologist who is available to provide support. Just let your team know if you would like to meet the psychologist.