Course Introduction

This course has been designed as an introduction to the speciality of caring for children and
adolescents with cancer (paediatric and adolescent oncology). The course provides an
overview of many different aspects of caring for young people with cancer - physical,
emotional and psychological.

You will be introduced to the key types of cancer affecting young people and to their
treatments; to some of the important social and emotional issues that are present with
children with cancer and to the effect that childhood cancer can have on family life. You will
also be encouraged to reflect upon how the care of young people with cancer is organised.

Childhood and adolescent cancer is rare and the care is often undertaken in a number of
settings. Within this course, an emphasis is placed on the importance of maintaining
normality throughout treatments so that a child or adolescent with cancer may be able to
continue to attend school or work throughout their treatment. With increasing survival rates,
more people will come into contact with a childhood/adolescent cancer survivor. As the
visibility of children and adolescents with cancer increases (partly because of the increase in
survival rates) so the need for an insight into their care becomes more important.

Learning outcomes
At the end of the course, learners will be able to:

describe the key causes and the incidence of cancer in children and young people;

discuss the main types of cancer in children and young people;

understand the treatment approaches to childhood/adolescent cancers;

consider the range of side effects attributable to childhood/adolescent cancer and its
treatments;

demonstrate an understanding of the psychosocial issues related to cancer in children

and young people;

describe how cancer care for children and young people is organised in their country;

name their key local and national childhood cancer organisations and treatment
centres.

Wherever possible, information from a broad range of countries is provided. However, a truly
international coverage is impossible, particularly in an introductory course like this one.
Although there are a lot of similarities between countries when discussing childhood cancer
and cancer care, there are also many differences (particularly in the way childhood cancer
care is organised).

Many of the optional activities in the course ask you to undertake some research to find
relevant information and statistics about childhood cancer and cancer care organisations in
your region. If you wish to gain the greatest benefit from this course you should attempt at
least some of these activities. We advise you to discuss ideas with your colleagues and also to
use the internet extensively.

Statistics on the incidence of cancer in your country are usually made freely available on the
websites of national organisations. You may also find that the key childhood cancer-related
organisations in your country provide a fantastic range of supplementary material. (A number
of useful websites and organisations to help you in your research are highlighted in the
'Resources' section available from the right-hand menu of this course.)

Definitions
A number of different definitions are used throughout the world to identify infants, children,
adolescents and young people. We have attempted, wherever possible, to use the following
within this course:

Infants - those aged between 0-5 years.

Children - those aged between 0-18 years.
Adolescents - those aged between 10-19 years.
Young people - those aged between 10-24 years.

Note: A range of statistics about cancer in children and young people are presented in this
course. The use of different age group definitions by cancer registries makes international

comparisons of the incidence of cancer difficult. Wherever possible we have highlighted the
age-group definition used by the source.

Course Introduction : Activity

This is an optional activity.

The aim of this activity is to help you assess your existing knowledge of cancer in children
and young people.

Before progressing to Module 1, think about and perhaps make notes in response to the
questions below. You can keep a record of your reflections on this activity in your personal
Learning Log (available from the right-hand menu).

1.

How much experience do you have of caring for a child with cancer? If you have cared
for children with cancer, try to remember one particular case. What type of cancer did
this particular child have and how was it treated? What was the outcome?

2.

How would you rate your knowledge of the treatments for cancers in children and
young people?

3.

How familiar are you with the way in which care for children and young people with
cancer is delivered in your region or country? Can you identify specialist treatment
centres close to you? What services do they offer? If a child you know was diagnosed
with cancer, do you know what would happen to them? Who would look after the child,
and where?

Course Structure

Where do I begin?
There is no single route through this course that you have to take. There is no timetable and
there are no deadlines. You can work through the materials at your own pace and in
whatever order you choose.

However ....
the course and the learning environment are very carefully organised. The principal course
content (which is accessed from the left-hand menu) is structured into Modules (about
specific themes and specific aspects of cancer in children and young people). Within each
Module there are a number of Units (which focus on specific topics).

If you follow the course content in order from Module 1 to Module 7, you will begin with a
general introduction to cancer and its causes and incidence in children and young people
(Module 1). You are then presented with introductions to each of the major childhood cancers
(Module 2). Module 3 provides a brief overview of the various ways in which cancers are
diagnosed and an introduction to staging - the measurement of how advanced a cancer is.
Module 4 examines the key treatment options for cancer in children and young people and
the late effects of childhood cancer treatment. Module 5 focuses upon the psychological and
social effects of cancer and cancer treatment upon children and their families generally and it
also provides an introduction to the specific effects upon, and the specific needs of,
adolescents with cancer. An example of the way in which cancer care is organised for
childhood cancer patients in the UK is presented in Module 6 and the course is summarised
and concluded in Module 7.

Self-assessment and activities

At the end of most Units you are presented with an opportunity to assess yourself on your
knowledge and understanding of the material. The majority of these assessments are
multiple-choice tests. On completion of a test, you submit your answers and within seconds
you are presented with your score and with feedback for each of your 'correct' answers.

By undertaking all of the tests and the course evaluation, you are deemed to have
'completed' the course. Your Learning Record will reflect the number of tests you have taken
and an overall score for the course. On completion of the course, you will gain access to a
'Record of Achievement' and a certificate that you can print and keep in a learning portfolio.

Note: you can retake the tests in the course as many times as you like in order to improve
your overall course score. The highest score for each test will always be used to determine
your overall score.

Each Module also presents you with a number of optional learning activities. These activities
present you with questions and issues to think about and research. They also invite you to
record them in your personal Learning Record (available from the right-hand menu).

Alternative routes through the course materials

While there is a lot of value to be gained from progressing through the Modules in order,
there are other learning materials you can access and other routes you can take through the
course.

You could simply work through all of the tests. The 'Tests' link in the right-hand menu will
take you to an index of all of the available tests.

You might like to read about and access the external resources described and linked to from
within the course. The 'Resources' link in the right-hand menu collects together a wealth of
further resources.

You could read the answers to frequently-asked questions or even submit a question to our
course expert, via the 'Ask the expert' page, and have your question considered for inclusion
on the FAQs page.

These and other routes and resources are available to you as part of this course. Follow your
own paths; retrace your steps; explore other directions.

Please let us know what you think of the course by completing an evaluation form online to
help us continually improve this course and its learning site.

Module 1 : Cancer and Children

This Module provides a brief introduction to what cancer is along with an overview of both the
causes and incidence of cancer amongst children and young people.

Learning outcomes
At the end of this Module, learners should be able to:

explain what cancer is;

discuss the trends regarding the incidence of cancer in childhood;

describe the major causes of cancer in children and young people.

Module 1 : Contents

Unit 1 : Cancer

Unit 2 : Incidence and Survival of Cancer in Childhood

Unit 3 : Causes of Cancer in Children and Adolescents

Unit 1 : Cancer
The word 'cancer' comes from the Latin for 'crab' or 'crayfish' and was first used by
Hippocrates to describe the swollen veins radiating from a breast cancer. Cancer is not one
disease but a collection of different pathologies affecting different parts of the body. The
individuality of each person and the variety of pathologies are reflected in the varieties of
treatments and outcomes. Although the experiences and paths followed by individuals with
cancer will differ greatly, there are some similarities in the way cancer develops and spreads
within the body.

Cancer can be described as a group of diseases in which cells grow uncontrollably and have
the ability to spread away from the site of origin. Each type of cancer has its own
characteristics. When a cancer spreads to another part of the body it takes its own
characteristics with it. So, for example, if Wilms' tumour spreads to the lung the tumour in
the lung would both look and behave the same way as the original tumour. This is how
primary tumours that have not been located can be found by examining the secondary
tumour. Cancer cells develop as a result of damage to DNA, the controlling mechanism for all
cell activities. This damage may be caused by a multitude of factors including environmental,
dietary and genetic factors. Damaged DNA is normally repaired by the body, but in cancer
cells this does not occur.

It is now believed that cancers originate from a single cell. This cell divides and eventually
forms what we would class as being a tumour. For a tumour to be recognised by either clinical
or radiographic examination it is likely to contain about 1 billion cells.

Tumours can be either malignant or benign. Malignant tumours are usually cancerous and
have a number of characteristics different to that of normal cells. The rate of reproduction in
cancer cells is greater than the rate of cell death generally. This is in contrast to normal cells
where the number of cells produced equals those dying. Cancers do not always grow
particularly fast. It is actually the lack of cell death in cancers that causes them to grow
rather than the speed of reproduction being particularly fast. Another important characteristic
of cancer cells is their inability to recognise when they come into contact with different cell
types. This means they are able to spread away into surrounding tissue and other areas of
the body (metastases). Cancerous tumours can originate in any of the common tissue types
in the body. Contrary to popular belief, it is only tumours that arise in the epithelium, such as
bowel and oesophageal cancers, that are termed 'carcinomas'. However, very few children get
carcinomas of this sort.

Benign tumours are not classified as cancers. The main difference between benign and
malignant (cancerous) tumours is that benign tumours do not attempt to spread away from
the site of origin.

1.1 - Cancer
Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

In cancer cells, the rate of cell reproduction is greater than the rate of
cell death.
This statement is:
o

True

False

2.

Tumours that do not spread away from their origin are called "Benign"
tumours.
This statement is:
o

True

False

3.

The controlling mechanism for all cell activity is the:

o

Cytoplasm

Cell Membrane

PSA

DNA

4.

Do cancers always grow very quickly?

o

Yes

No

Submit Answ ers

Unit 2 : Incidence and Survival of Cancer in Childhood

Cancer remains the second leading cause of death in children in the developed world. The
latest statistics available from the 1990s demonstrate that there were 140 per million children
(aged under 18) and 157 per million young people (between the ages of 10 and 24)
diagnosed with cancer annually.

Within developed countries there are a number of common statistical 'themes' regarding
cancer in childhood:

cancer is very rare, generally comprising less than one percent of all cancer
registrations;

incidence levels remain static;

mortality rates have decreased over the last ten to twenty years, reflecting improved
diagnosis and treatments;

cancer is one of the most common causes of death in this age group (after death
because of accidents and congenital abnormalities);

cancer presentation can be linked to paediatric 'growth spurts'.

The picture for many developing countries is quite different. For example, in some areas,
malnutrition, AIDS, malaria and tuberculosis are the major causes of childhood mortality.
Furthermore, for some areas of the world it is difficult to access accurate statistics on cancer
in childhood.

The types of cancer that children are diagnosed with are, on the whole, different to those in
adults. The age at presentation varies with the different types of disease. For example:

leukaemia affects children of all ages;

neuroblastoma and Wilms' tumour are most common in the first five years of life;

Hodgkins disease and bone tumours have their peak incidence in adolescence and
early adult life.

International trends and statistics

Below, we provide a summary of trends and some statistics on childhood cancers for five
developed countries: United Kingdom, United States, Canada, Australia and New Zealand.
Please note that, whilst these stand alone in their own right, comparisons need to be
undertaken carefully as, results vary. For example, some tables look at ages 1-14 and some
at ages 1-20, and so on.

United Kingdom
In the UK, cancer is the third most common cause of death after accidents and congenital
abnormalities. One in 650 children are diagnosed with cancer annually, giving rise to 1700
new cases a year in children aged 0-15. Cancer is 20% more common in boys than in girls.
Whereas in the 1960s the long term survival rate was approximately 35%, the advances

made in treatments now give an overall survival rate of 75% with a range of 45% to 90%
across all tumour types.

Table 1, below, presents a contemporary picture of the numbers and types of childhood
cancers diagnosed in the UK each year. (Source: CancerBacup.)

Cancer Type

Cases per Year

Percentage (%)

Leukaemia

440

30.1

Brain tumours

330

22.6

Neuroblastoma

100

6.8

Non-Hodgkin's lymphoma

90

6.2

Wilms' tumour

80

5.5

Rhabdomyosarcoma

70

4.8

Hodgkin's lymphoma

60

4.1

Retinoblastoma

50

3.4

Germ cell tumours

50

3.4

Osteosarcoma

40

2.7

Ewing's sarcoma

40

2.7

Langerhan's cell Histiocytosis

40

2.7

Liver tumours

20

1.4

Others

50

3.4

Total

1460

100

Table 1: Types and numbers of childhood cancers diagnosed in the UK.

Figure 1, below, produced by the Office for National Statistics in the UK, demonstrates the
trends in five-year survival rates for children diagnosed with leukaemias and lymphomas.

Figure 1 provides one indication of how mortality rates have dropped dramatically since the
1960s.

Figure 1: Trends in five-year survival rates for children diagnosed with leukaemias and
lymphomas from 1962 to 1996.

United States
The picture in the United States is similar to that in the UK: leukaemia is the most common
childhood cancer, and the overall incidence of cancer in childhood has remained fairly
constant whilst the mortality rate has dropped significantly. The tables and figures below
provide a picture of the incidence and mortality rates for the different types of childhood
cancer and a picture of childhood cancer incidence and mortality rates generally.

Site

Male

Female

Mean

All sites

15.5

14.1

14.8

Leukaemia

4.8

4.2

4.5

Acute Lymphocytic Leukaemia

3.8

3.4

3.6

Brain / ONS

3.5

3.1

3.3

Soft tissue

1.0

1.0

1.0

Non-Hodgkin lymphoma

1.3

0.6

0.9

Kidney and renal pelvis

0.8

1.0

0.9

Bone and Joint

0.8

0.6

0.7

Hodgkin lymphoma

0.6

0.5

0.6

Table 2: Cancer Incidence Rates per 100,000* in Children 0-14 Years, By Site, 1997-2001.
* Age-adjusted to the 2000 US standard population.
ONS = Other nervous system

Site

Male

Female

Mean

All sites

2.7

2.2

2.5

Leukaemia

0.9

0.7

0.8

Acute Lymphocytic Leukaemia

0.4

0.3

0.4

Brain / ONS

0.7

0.7

0.7

Non-Hodgkin lymphoma

0.1

0.1

0.1

Soft tissue

0.1

0.1

0.1

Bone and Joint

0.1

0.1

0.1

Kidney and Renal pelvis

0.1

0.1

0.1

Table 3: Cancer Death Rates per 100,000* in Children 0-14 Years, By Site, 1997-2001.
* Age-adjusted to the 2000 US standard population.
ONS = Other nervous system

Figure 2: Cancer Incidence & Death Rates in Children 0-14 Years, 1975-2001.
* Age-adjusted to the 2000 US standard population.

Figure 3: Trends in Survival, Children 0-14 Years, All Sites Combined, 1974-2000.
*5-year relative survival rates, based on follow up of patients through 2001.

Canada
The quotation below, from a report by The Canadian Childhood Cancer Surveillance and
Control Program, underlines how rare childhood cancer is and outlines the most common
cancers affecting different groups of children and young people.

"In 2002, of nearly 8 million Canadian children and adolescents younger than 20 years of
age, an estimated 1,300 were diagnosed with cancer.

Among infants, the most common cancers are neuroblastomas (22 percent) followed by
leukaemias (17 percent), central nervous system tumours (13 percent) and retinoblastoma
(11 percent).

The most common cancers for children between the ages of one and four are leukaemias (43
percent), central nervous system tumours (16 percent), followed by neuroblastoma and
Wilms' tumour (both 9 percent).

Lymphomas are the third most common cancer (12 percent) in the five to nine year age
group after leukaemias (32 percent) and central nervous system tumours (30 percent).

In the 10 to 14 year age group, cancers of the central nervous system are the most common
(25 percent), followed by leukaemia (23 percent) and lymphomas (20 percent).

In the 15 to 19 year age group, the most common cancers were lymphomas (29 percent),
carcinomas (19 percent), followed by germ cell tumours (15 percent) and leukaemia (11
percent).

A higher number of boys than girls are diagnosed with most cancer types. However, girls
outnumbered boys in the categories of carcinomas, renal tumours and other and unspecified
malignant neoplasms."

(Source: The Canadian Childhood Cancer Surveillance and Control Program (CCCSCP) - Public
Health Agency of Canada.)

Australia
The quotation below, from a report by the Australian Bureau of Statistics, provides a picture
of the incidence and mortality rates that is similar to that found in the UK and the US.

"Childhood cancer is relatively uncommon (children aged 0-14 years made up less than 1% of
all cancer patients in each year from 1983 to 2000). However, cancer is a leading cause of
death among children aged 1-14 years (118 children in this age group died from cancer in
2002). Death rates for children improved from four deaths per 100,000 children aged 0-14
years in 1993, to three per 100,000 in 2000, reflecting advances in various cancer
treatments.

Cancer incidence is lowest in late childhood and increases with age. The most common forms
of cancer among children aged 0-14 years are lymphatic leukaemia, and brain and central
nervous system cancers, which together accounted for 51% of cancer incidence in this age
group in 2000. For almost all cancers, incidence is higher among boys than among girls."

(Source: Australian Bureau of Statistics.)

New Zealand
In New Zealand, cancer is the third most common cause of death amongst children following
congenital abnormalities and motor vehicle accidents.

"In 2000 there were 164 cancer registrations for children aged 14 years and younger,
representing 0.93 percent of registrations for all ages. The most common type of childhood
registration was leukaemia with 55 registrations (33.5 percent of all childhood cancer
registrations), followed by brain cancer with 28 registrations (17.1 percent of childhood
cancer registrations).

There were 36 childhood cancer deaths in 2000, accounting for 6.4 percent of total childhood
deaths. The most common cause of cancer death for children was cancer of the brain (50.0
percent of all childhood cancer deaths) with 18 deaths."

(Source: New Zealand Health Information Service.)

Permissions
Figure 1 is taken, with permission, from 'The Health of Children and Young People', Office for
National Statistics, 2004. The full volume is available via the National Statistics website.

Tables 2 and 3 and Figures 2 and 3 are from the Surveillance, Epidemiology, and End Results
Program, 1975-2001, Division of Cancer Control and Population Sciences, US National Cancer
Institute, 2004.

1.2 - Incidence and Survival of Cancer in

Childhood
Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

Which ONE of the following statements is most accurate?

o

Within developed countries, childhood cancers generally represent less

than one percent of all cancer registrations.

o

Within developed countries, childhood cancers generally represent less

than five percent of all cancer registrations.

Within developed countries, childhood cancers generally represent less

than ten percent of all cancer registrations.

Within developed countries, childhood cancers generally represent less

than twenty percent of all cancer registrations.

2.

Has the incidence of all childhood cancers in developed countries

increased greatly over the last thirty years?
o

Yes

No

3.

Mortality rates associated with childhood cancers have decreased in

developed countries over the last ten to twenty years.
This statement is:
o

True

False

4.

Childhood cancer is one of the most common causes of death in children

in DEVELOPED countries.
This statement is:
o

True

False

5.

Are children generally diagnosed with the same types of cancers as

adults?
o

Yes

No

6.

For some childhood cancers there is a strong relationship between the

age of the child and the type of cancer diagnosed.
This statement is:
o

True

False

Unit 3 : Causes of Cancer in Children and Adolescents

The causes of cancer in children and young people are generally different from the most
common set of causes of cancer in adults. It must also be noted that conclusive evidence for
many possible causes of childhood cancers has not been produced and much research is still
being undertaken. Obtaining statistical significance of causes and clusters is often extremely
difficult because of the small numbers of children who develop cancer. Below, we list and
discuss some of the most common causes of cancer in children and young people.

1. Family history
There is evidence that children born into a 'cancer family' are at higher risk than those who
are not. In addition, specific links between adult and child cancers are now identified; for
example, between breast cancer in adults and Wilms' tumour in the children of women with
breast cancer.

2. Presence of genetic defects in the child

The link between other genetic defects and cancer in children has been identified; for
example, the risk of leukaemia increases in children with Down's syndrome.

3. Use of chemotherapy drugs

The rising incidence of second tumours as a result of exposure to chemotherapeutic agents is
now established.

4. Exposure to ionizing radiation

Environmental exposure, such as that experienced after the 1986 Chernobyl nuclear plant
disaster, in the Ukraine, demonstrates that excessive exposure to ionizing radiation may lead
to an increase in cancer in children and young people.

"Many health effects have been observed that could be attributed to the accident. Most
significantly, a notable rise in thyroid cancer in the most contaminated areas of the former
Soviet Union is most likely a direct result of the accident. The cancer is most prevalent in
children who were aged between 0-5 years at the time, and is thought to be caused by
radioactive iodine contamination in certain foods and milk collecting in the thyroid gland.
Children, understandably, since they drink more milk and have smaller thyroid glands,
received a higher dose than adults. The disease is treatable and not often fatal. Although the
risk has subsided as the iodine has decayed to low levels, the disease takes years to show up
and the number of cancers diagnosed is still increasing.

"Conversely, there have been no observed increases (to date) in rates of leukaemia,
congenital defects, abnormal pregnancies or any other radiation-induced sickness, even in
heavily contaminated areas. Estimates of the total lifetime cancers expected as a result of the
accident are 0.01% above the natural incidence in Europe, and only 0.004% in the Northern
hemisphere overall. Contaminated areas are considered to be those with Cs-137 deposition
levels greater than 37kBq/m2. These areas are restricted to Ukraine, Belarus and Russia, but
cover about 3% of the European part of the former Soviet Union and about five million people
are still living there."

(Source: BBC - The Chernobyl Disaster.)

5. Environmental factors
There is some evidence that certain environmental factors may contribute to the development
of cancer in children. However, it should be noted that many of the adult cancers caused by
environmental factors are linked to the length of time, and amount, of exposure, whereas
generally children have not experienced that length or amount of exposure. However,
behaviours such as sunbathing and unprotected sex are responsible for cancers in young
people and other behaviours such as smoking are risk laden for later life.

Tobacco related exposure

There is some evidence to suggest that children who are exposed to tobacco smoke in the
home or whose parents have been long-term smokers have an increased risk of developing

cancer as a child. There is also emerging evidence of the role of infections (viruses and
bacteria) in the development of childhood cancers.

Exposure to non-ionizing radiation

Non-ionizing radiation is low-frequency radiation that does not carry enough energy to ionise
living material. Mobile phones and electricity power lines are examples of everyday 'objects'
that emit this type of radiation. There is much controversy surrounding non-ionising radiation
as a cause of cancer, and to-date there is no conclusive evidence that these cause cancer.

6. Socio-economic factors
For a number of childhood cancers there appears to be a link between socio-economic status
and type of cancer. The Office for National Statistics in the UK reported the following in their
publication, 'Health of Children and Young People' (2004):

"In Britain, the incidence of ALL [acute lymphoblastic leukaemia] is higher in areas of higher
socio-economic status, particularly in early childhood. The lower incidence among Black
children in the United States of America (USA) and Africa may be at least partly a social class
effect. A higher incidence of Hodgkin's disease in older children and young adults has been
associated with higher socio-economic status in several studies. In many developing
countries, Hodgkin's disease has a higher incidence among young children and this pattern
seems to be linked to poor socio-economic conditions. Neuroblastoma may be slightly more
common in children of lower socio-economic status."

Module 2 : Types of Childhood Cancer

This Module will provide an overview of the most common cancers that children may be
diagnosed with.

Generally, it is important to understand that childhood cancer is rare, accounting

for 0.5% of all cancers. In the UK, for example,

1 child in 650 develops cancer by 15 years of age;

there are 120-150 new cases per million children aged under 15 per year; that is,
about 1500 cases per year.

In Britain childhood cancer incident rates have increased by over 40% since the late
1960s, the reason for this is poorly understood, though improvements in diagnosis and
registration are likely to have played a part.

The types of malignant disease that children are diagnosed with are, on the whole, different
to those in adults. The age at presentation varies with the different types of disease. For
example,

leukaemia affects children of all ages;

neuroblastoma and Wilms' tumour are most common in the first 5 years of life;

Hodgkin's disease and bone tumours have their peak incidence in adolescence and
early adult life.

Learning outcomes
At the end of this Module, learners will be able to:

identify the most common childhood cancers;

discuss the symptoms of each of the most common childhood cancers;

describe how each of the cancers is diagnosed;

highlight the key treatment for each type of cancer.

Module 2 : Contents

Unit 1 : Leukaemia

Unit 2 : Brain Tumours

Unit 3 : Lymphomas

Unit 4 : Neuroblastoma

Unit 5 : Soft Tissue Sarcomas

Unit 6 : Wilms' Tumour (Nephroblastoma)

Unit 7 : Bone Tumours

Unit 8 : Retinoblastoma

Module 2 : Activity
This is an optional activity.

After completing all of the Units in this Module you should make your own brief notes on each
of the major types of childhood cancer using the following headings:

Description of cancer type

Symptoms

Method(s) of diagnosis

Treatment options

For each of the types of cancer covered in this Module you should also attempt to find
statistics on their incidence in your region or country. If a specific cancer which is one of the
common cancers in your region or country is not covered in this Module, then you should try
to find out more information about it and write notes on it using the above headings. You will
find the addresses of a number of useful websites in the Resources section available from the
right-hand menu.

You can keep a record of your findings about, and reflections on, this activity in your personal
Learning Log (available from the right-hand menu).

Unit 1 : Leukaemia
Leukaemia is the most common type of childhood cancer in the developed world, accounting
for about 30% of all cancers in this age group. It is a group of blood diseases characterised
by a rapid increase in the number of immature white blood cells in the blood-forming tissues.

Leukaemia starts when bone marrow stem cells, which produce the red and white blood cells
that carry oxygen and fight infection respectively, start to produce abnormal white blood
cells. (The word leukaemia is derived from 'leuko' (white) and 'cytes' (mature blood cells).)

Leukaemia is divided into two groups - acute and chronic - and is classified according to the
type of abnormal cell found most in the blood: lymphoid cells or myeloid cells. When

leukaemia affects lymphoid cells, it is called lymphocytic leukaemia. When myeloid cells are
affected, the disease is called myeloid or myelogenous leukaemia.

Acute leukaemias can appear and progress suddenly and require urgent treatment.

Chronic leukaemias usually take much longer to form (over months and sometimes years)
and can be monitored so that when treatment is given it is of the most appropriate type.

80% of children with leukaemia will have acute lymphoblastic leukaemia (ALL), which is also
known as acute lymphocytic leukaemia. Most of the remainder will have acute myeloid/acute
non-lymphocytic (AML/ANLL) leukaemia. Chronic myeloid leukaemia (CML) and other
myeloproliferative disorders are rare.

Symptoms
When bone marrow and other organs are affected by leukaemia blast cells then clinical signs
and symptoms present themselves. The most common signs and symptoms are listed below.

Clinical signs

Abnormal blood cell count, low haemoglobin and platelet count

Evidence of circulating blast cells.

Symptoms

Malaise - due to suppressed or dysfunctional red blood cells

Infections - due to suppressed or dysfunctional white blood cells

Abnormal bruising - due to reduced blood platelets

Hepatosplenomegaly - simultaneous enlargement of both the liver (hepatomegaly)

and the spleen (splenomegaly) due to presence of excessive white blood cells

Lymphadenopathy - swelling of one or more lymph nodes due to presence of

excessive white blood cells

Bone pain - may occur if the cancer has spread to this area due to presence of
excessive immature blood cells.

Diagnosis
Blood and bone marrow tests are essential for accurate diagnosis and to obtain
immunological and cytogenetic markers which give useful prognostic information.

Treatment
The core treatment for leukaemia is chemotherapy, sometimes with the addition of radiation
therapy.

Bone marrow transplantation is used for some children who have relapsed or are of high risk
of relapse in first remission. Tissue damaged by the treatment is rebuilt by introducing
healthy bone marrow.

Prognosis
Prognosis in acute lymphoblastic leukaemia (ALL) is related to age, white cell count at
diagnosis, presence or absence of specific genetic markers and speed of response to initial
treatment. 8 out of 10 children diagnosed with ALL will be cured.

2.1 - Leukaemia
Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

Which ONE of the following statements is most accurate?

o

Leukaemia accounts for around 10% of all childhood cancers in the

developed world.
o

Leukaemia accounts for around 30% of all childhood cancers in the

developed world.

Leukaemia accounts for around 50% of all childhood cancers in the

developed world.
Leukaemia accounts for around 70% of all childhood cancers in the

developed world.

2.

Where in the body does leukaemia originate?

o

The bladder

The brain

The kidneys

Bone marrow

3.

When leukaemia affects lymphoid cells it is called myelogenous

leukaemia.
This statement is:
o

True

False

4.

Which ONE of the following statements is true?

Generally, chronic leukaemia appears rapidly and requires urgent

o
treatment.

Generally, chronic leukaemia can take months or years to form and

can often be monitored closely so that the most appropriate treatment is provided.

5.

Which ONE of the following statements is true?

Generally, acute leukaemia forms gradually and over a long period of

o
time.
o

Generally, acute leukaemia can appear and progress suddenly,

requiring urgent treatment.

6.

The most common form of leukaemia in childhood is:

Acute myeloid leukaemia

Chronic myeloid leukaemia

Acute lymphoblastic leukaemia

Chronic lymphoblastic leukaemia

7.

Which ONE of the following statements is most accurate?

o

The clinical signs and symptoms of leukaemia result from abnormal

cells in the brain.

o

The clinical signs and symptoms of leukaemia result from infiltration of

the nervous system with leukaemia blast cells.

o

The clinical signs and symptoms of leukaemia result from infiltration of

the bone marrow and other organs with leukaemia blast cells.

8.

Which ONE of the following is NOT a CLINICAL SIGN of leukaemia?

o

Low haemoglobin count

Low platelet count

Bone and joint pain

Abnormal blood cell count

9.

Which ONE of the following is NOT a SYMPTOM of leukaemia?

o

Abnormal bruising

Bleeding

Malaise

Infections

10.

Which ONE of the following is normally required in order to confirm a

diagnosis of leukaemia?

A CT scan

A PET scan

Blood and bone marrow tests

Radiotherapy

11.

Which ONE of the following is the key treatment for leukaemia?

Radiotherapy

Chemotherapy

Blood stem cell transplant

Surgery

12.

What percentage of children with acute lymphoblastic leukaemia are

likely to be cured?

20%

40%

60%

80%

Submit Answ ers

Unit 2 : Brain Tumours

A brain tumour is an intracranial mass created by an abnormal and uncontrolled growth of
cells. Brain tumours are the second most common type of cancer in children. Brain and
central nervous system tumours account for 25% of childhood cancers. Unfortunately they
remain a challenge to treat with higher rates of morbidity than for other childhood cancers.
Brain tumours in children are usually the primary site of tumour, compared with adult brain
tumours which are more commonly secondary tumours or metastases.

In contrast to other types of cancer, primary brain tumours rarely spread. Although they
never spread, benign brain tumours also cause problems as they can grow and increase
intracranial pressure.

Brain tumours can present at any age in childhood and generally occur more in boys than
girls.

The two most common types of childhood brain tumour are:

Gliomas (astrocytomas and ependymomas) - which arise from the supportive

tissue of the brain, and can be found in any part of the organ.

Medullablastomas - which develop in the cerebellum at the back of the brain.

Symptoms
Children can present with a variety of symptoms depending on the type, site and size of
tumour within the brain, including:

headaches

vomiting (sometimes without nausea)

altered state of consciousness (somnolence, coma)

dilatation of the pupil on the side of the lesion (anisocoria)

visual disturbances

seizures

cranial nerve disorders.

Diagnosis
Diagnosis is made following careful examination of the central nervous system. Imaging,
using CT and MRI scans, is essential and a biopsy may be taken. A brain biopsy is conducted
by removing part of the skull and using a needle to remove a sample of the tumour tissue
(CT and MRI scans are used to identify the exact location of the possible tumour prior to
biopsy).

Treatment
The mode of treatment depends on the type and site of tumour and on the age of the child.

Surgery
Surgery is often the first line of treatment and usually aims to remove as much of the tumour
as possible with the least amount of damage to intact brain function as possible. A shunt or
ventriculostomy may be performed to relieve hydrocephalus (the accumulation of
cerebrospinal fluid within the skull).

Radiotherapy
Some brain tumours are more radio-sensitive than others. However, radiation can cause
devastating side effects on the developing brain, therefore treatment is limited to children
over the age of 5 if possible, to help reduce long-term effects.

Chemotherapy
Chemotherapy is used increasingly to treat children of all ages with a CNS tumour. A variety
of drugs are used and these can also be given to infants in an attempt to delay tumour
growth until the child is old enough to receive radiotherapy.

Steroids
Steroids may also be given as they can help to reduce the swelling that often surrounds brain
tumours. The steroids do not treat the tumour but can improve symptoms. They may be
given before or after surgery, or during or after radiotherapy.

Prognosis
If the nodes are localised to the neck, prognosis is related to the success of surgery (whether
the tumour was completely removed rather than cancer cells remaining) the type of tumour,
and its location.

Late effects
In the long term, children who have received treatment for a brain tumour can suffer from a
variety of late effects along a continuum of severity. Effects can be physical, cognitive,
neurological or endocrinological, or all four. Close follow up is therefore essential to monitor
progress, provide support and for early detection of relapse or secondary malignancies.

2.2 - Brain Tumours

Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

Which ONE of the following statements is most accurate?

o

Brain tumours are the most common type of cancer in children.

Brain tumours are generally the second most common type of cancer

in children.
o

Brain tumours are the rarest type of cancer in children.

Brain tumours do not present during childhood.

2.

Are brain tumours in children usually primary or secondary tumours?

o

Primary

Secondary

3.

4.

Which ONE of the following statements is most accurate?

o

Brain tumours in childhood generally occur more in boys.

Brain tumours present equally in boys and girls.

Brain tumours in childhood generally occur more in girls.

Gliomas can occur anywhere within the brain.

This statement is:

True

False

5.

Which ONE of the following would NOT usually be considered a symptom

of brain tumour?
o

Altered state of consciousness

Visual disturbances

Bone and joint pain

Vomiting (sometimes without nausea)

6.

Which ONE of the following statements is true?

o

Biopsy IS USED for determining brain tumours.

Biopsy IS NOT USED for determining brain tumours.

7.

The first line of treatment for brain tumours is:

o

Chemotherapy

Radiotherapy

Surgery

Blood stem cell transplantation

8.

Which ONE of the following statements is most accurate?

o

Radiotherapy can be used for diagnosis of some brain tumours.

Radiotherapy is NOT usually a first-line treatment for brain tumours in

children under five years of age because it is ineffective.

o

Radiotherapy is usually the first-line treatment for brain tumours in

children under five years.

Radiotherapy is NOT usually a first-line treatment for brain tumours in

children under five years because of the possible devastating side effects on the
developing brain.

Unit 3 : Lymphomas
Lymphomas are malignancies of the lymphatic system, part of the immune system.
Lymphoma accounts for approximately 10% of childhood cancers. There are two main groups
of lymphomas: non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma (also know as
Hodgkin's disease, as it was first described by Thomas Hodgkin in 1832). NHL is more
common in childhood and refers to a group of closely related cancers that affect the white
cells of the lymphatic system. Hodgkin's disease is seen more frequently in adolescence.

Both non-Hodgkin's lymphoma and Hodgkin's disease begin in the lymph nodes. The key
difference between them is in the presence of abnormal cancerous cells called ReedSternberg cells in Hodgkin's disease. These are not present in NHL. A further difference is
that Hodgkin's disease tends to spread to adjacent lymph nodes and is less likely than NHL to
metastasise to other organs outside the lymphatic system.

Non-Hodgkin's lymphoma
In children, NHL can present wherever lymphoid tissue is present. There are two main types
of non-Hodgkin's lymphoma:

B-cell NHL usually affects the lymph nodes in the neck, head, throat and
abdomen.
T-cell NHL usually affects the lymph nodes in the neck and presents in the
mediastinum (chest).

Symptoms
The symptoms of NHL are usually dependent on the site of the tumour. A common presenting
feature is enlarged lymph nodes at the site of disease. The placement of the enlarged nodes
determines how the child presents. Other, vaguer, symptoms include:

Fever

Sweating (particularly night sweats)

Fatigue

Weight loss

Itchy or reddened skin

Diagnosis
X-rays and CT and MRI scans, are usually used in the initial testing stages for non-Hodgkin's
lymphoma. Biopsy remains the key method for accurate diagnosis.

Treatment
Chemotherapy is the main method of treatment and is sometimes used alongside
radiotherapy. Aggressive chemotherapy may require additional blood stem cell
transplantation.

Prognosis
If the nodes are localised (for example, cervical or oropharynx) the NHL is usually of B-cell
origin and the prognosis is good.

If the nodes are intrathoracic (within the cavity of the chest) with a mediastinal mass and a
pleural effusion (the accumulation of fluid in the pleural cavity which surrounds the lungs),
the NHL may be T-cell origin and the prognosis is good.

If there is intra-abdominal disease, this is typically advanced B-cell disease and treatment will
be very intensive, but the prognosis is now improving.

Hodgkin's disease

Symptoms
This usually presents with a painless lymphadenopathy (swelling of the lymph nodes), most
frequently in the neck (and on one side only). The lymph nodes are much larger and firmer
than the normal enlarged glands associated with childhood. Other, vaguer, symptoms include:

Fever

Sweating (particularly night sweats)

Weight loss

Irritable or reddened skin

Diagnosis
Biopsy, sometimes involving total resection of the lymph gland, remains the key method for
accurate diagnosis. X-rays and CT and MRI scans are used in the initial testing stages for
non-Hodgkin's lymphoma.

Treatment
Dependent on the stage of the disease, a combination of chemotherapy and radiotherapy is
used as treatment.

Prognosis
Overall, 80% of children will be cured although this decreases to 60% if they present with
widespread disease.

2.3 - Lymphomas
Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

In which type of lymphoma are Reed-Sternberg cells present?

Hodgkin's disease

Non-Hodgkin's lymphoma

2.

Within which group of young people is Hodgkin's disease most commonly

found?
o

New born babies

Infants (children aged under 5)

Children aged between 5 and 10

Adolescents aged between 10 and 19

3.

T-cell non-Hodgkins lymphoma usually affects the lymph nodes in the

_______.
The missing word is:
o

Head

Shoulder

Neck

Abdomen

4.

The key symptom of Hodgkin's disease is:

o

Swelling of the neck

Night sweats

Weight loss

Itchy (irritable) skin

5.

The key method of diagnosis for Hodgkin's disease and non-Hodgkin's

lymphoma is:
o

MRI

CT scan

X-ray

Biopsy

6.

Which ONE of the following is the most common treatment regime for
Hodgkin's disease and non-Hodgkin's lymphoma?
o

Surgery

Chemotherapy and / or radiotherapy

Bone marrow transplantation (BMT)

Peripheral blood stem cell transplants (PBSCT)

7.

The general prognosis for Hodgkin's disease and non-Hodgkin's lymphoma

is:
o

Good

Poor

Unit 4 : Neuroblastoma
Neuroblastoma is a disease that arises from the neural crest tissue of the adrenal gland and
sympathetic nervous tissue. It occurs most often in children under the age of five. Commonly,
the primary is of the adrenal gland (located above the kidneys). Most children present with
an abdominal mass. This mass is often large and complex, crossing the midline and
incorporating major blood vessels and lymph glands.

Symptoms
The symptoms of neuroblastoma are often vague and thus early-stage diagnosis can be rare.
This is why neuroblastomas diagnosed in children over two years of age have often already
metastasised (spread) at presentation, causing some or all of the following presentational
signs:

Pallor (pale appearance of the skin)

Weight loss

Hepatomegaly (enlargement of the liver)

Bone pain

Limp

Proptosis (forwardly displaced or 'bulging' eye)

Periorbital bruising ('black' or 'raccoon' eye)

Skin nodules.

Spinal cord compression (pressure on the spinal cord) may also be a clinical feature if there is
a para-vertebral tumour (tumour around the spine).

Diagnosis
Diagnosis can often be made from the presentational features but biopsy is usually taken. A
urine test may show raised urinary catecholamines and a specific scan may be used to assess
the extent of the disease (for example, an mIBG scan, involving the injection of
metaiodobenzyl guanidine which is taken up by neuroblastoma cells and is highlighted by
radiation scanning).

Treatment
The size, position and stage of the tumour, and the age of the child, dictate the treatment
offered.

Chemotherapy is usually the initial treatment, this may be followed by surgery or

radiotherapy. Children with wide spread disease will require intensive chemotherapy followed
by surgery, radiotherapy and possibly a new Immunotherapy treatment (using monoclonal
antibodies have shown to harness the bodys immune system and increase survival rates).

Prognosis
Unfortunately, the majority of children with neuroblastoma present with metastatic disease
and the prognosis for this disease remains poor. Only 30% of children can expect a cure.
However if disease is localised at diagnosis the prognosis is much improved. In young infants
this disease has been seen to spontaneously regress without intervention, becoming benign.

2.4 - Neuroblastoma
Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

Which ONE of the following is the most common site of neuroblastoma

primary tumours?
o

The lymph gland

The parathyroid glands

The adrenal gland

The thyroid gland

2.

Neuroblastoma is most common in which ONE of the following age

groups?
o

0 - 4 years

5 - 9 years

10 - 14 years

15 - 19 years

3.

The symptoms of neuroblastoma are easily detected, ensuring early

diagnosis.
This statement is:
o

True

False

4.

Which of the following symptoms would you NOT normally associate with
neuroblastoma?
o

Bone pain

Enlargement of the liver

Pallor (pale appearance of the skin)

Hydrocephalus

5.

Which ONE of the following is usually the initial treatment for

neuroblastoma?
o

Chemotherapy

Radiotherapy

Biopsy

Surgery

6.

Which ONE of the following statements about the prognosis for

neuroblastoma is most accurate?
o

Generally, around 10% of children can expect to be cured.

Generally, around 30% of children can expect to be cured.

Generally, around 50% of children can expect to be cured.

Generally, around 70% of children can expect to be cured.

7.

Neuroblastoma is one of the few cancers that can spontaneously regress

without intervention.
This statement is:
o

True

False

Unit 5 : Soft Tissue Sarcomas

Sarcomas (derived from the Greek word meaning 'fleshy growth') are malignant tumours of
the tissues that connect, support, or surround other structures and organs of the body.
Examples include: muscles, tendons, fibrous tissues, fat, blood vessels, nerves, and synovial
tissues (tissues around joints). Some soft tissue sarcomas are benign and, while not usually

life-threatening, their growth can overwhelm organs nearby and interfere with bodily
functions.

Bone tumours (often called osteosarcomas) are a type of sarcoma, but differ clinically from
soft tissue tumours and are treated differently. This is discussed in more detail in Unit 7.

The most common soft tissue sarcoma is rhabdomyosarcoma, developing from muscle, fat,
blood vessels and any other tissue that supports, surrounds and protects the bodys organs.
It accounts for approximately 4% of childhood cancers. Most commonly seen between the
ages of 2-6 years old. Other types of soft tissue sarcomas that affect children are:

Liposarcoma (originating in fat tissue)

Synovial sarcoma (originating in synovial tissue, including linings of joint cavities and
tendon sheaths)

Fibrosarcoma (originating in fibrous tissue)

The highest peak incidence of sarcomas is in the first few years of life.

Symptoms
The signs at presentation of rhabdomyosarcoma are dependent on the tumour's primary site
of origin. The most common sites of origin are head and neck, and genitourinary.

The most common head and neck symptoms are:

Proptosis (forwardly displaced or 'bulging' eye)

Nasal obstruction

Blood stained nasal discharge.

The common genitourinary (bladder or testes) symptoms are:

Dysuria (painful or difficult urination)

Urinary obstruction

Blood stained vaginal discharge.

Diagnosis
The most common test to diagnose rhabdomyosarcoma is a biopsy (usually undertaken under
general anaesthetic in children). A range of further tests (including CT and MRI scan, X-ray,
and blood and bone marrow tests) may be undertaken to ascertain the exact position, size
and stage of the cancer.

Treatment
Treatment depends on the site, size and extent of the disease. Surgery may be the first
option, with additional chemotherapy or radiotherapy. In the majority of cases aggressive
chemotherapy is required.

Prognosis
Overall cure rates are approximately 65%, although this is dependent on size and spread at
presentation. Metastatic disease (characteristically of the lung, liver, bone or bone marrow)
present in about 15% of patients, is associated with a poor prognosis.

2.5 - Soft Tissue Sarcomas

Try to answer all of the questions and when you are finished click the 'Submit Answers'
button. Your results will then be marked by the computer and shown to you. You can retake
this test as often as you like.

1.

2.

Which ONE of the following is NOT classed as a soft tissue sarcoma?

o

Fibrosarcoma

Liposarcoma

Osteosarcoma

Rhabdomyosarcoma

Where does rhabdomyosarcoma most commonly occur?

In the head and neck

In the bladder

In the testes

In the bowel

3.

Which ONE of the following is the key diagnostic method for

rhabdomyosarcoma?
o

Biopsy

X-ray

Blood or bone marrow test

CT or MRI scan

4.

Surgery alone is the most common treatment for rhabdomyosarcoma.

This statement is:
o

True

False

5.

Generally, what is the overall cure rate for rhabdomyosarcoma?

o

10 - 20%

30 - 40%

60 - 70%

80 - 90%