Overview

What is Paediatric Oncology?


Paediatric oncology is the research and treatment of cancers in children and young adults. Paediatric oncologists study and train in either paediatrics or oncology or both.


How common are cancers in childhood?


Although cancer in children is rare, it is the leading cause of death by disease after infancy among children in the World. According to a recent publication, it is estimated that 4,16,500 children and adolescents between the ages of 0 to 19 were diagnosed with cancer and 142,300 have died of this disease in the year 2017 (GBD 2017, Childhood Cancer Collaboration). The number of survivors continues to increase, given that the incidence of childhood cancer has been rising slightly in recent decades and that survival rates overall are also improving.


Speciality groups related to Paediatric oncology

Paediatrichemato-oncologists, paediatric surgeons, paediatric radiation oncologists, paediatric nurses, paediatric counsellors and other specialists such as endocrinologists, ophthalmologists, infectious disease specialists, intensivists, orthopaedic surgeons, psychologists, physiotherapists, etc. constitute the specialists dealing with children and adolescents with tumours/cancers.


What are the types of cancers noted in childhood?

Most childhood cancers fall into one of several specific types, as listed below followed by approximate incidences in India. (Cancer Atlas India)


  • Leukemia - 34.1%
  • CNS tumours - 12.4%
  • Lymphoma - 12%
  • Neuroblastoma - 3.2%
  • Soft-tissue sarcoma - 4.5%
  • Other neoplasms - 21%

Overall, among children and young adults between the ages of 0-19 years, the most common types of cancers are leukemias, brain and central nervous system tumours, and lymphomas. Among children (0 -14 years), the most common types of cancer are leukemias, followed by central nervous system tumours, lymphomas, soft tissue sarcomas (such as rhabdomyosarcoma) neuroblastoma, and kidney tumours. Among adolescents (15-19 years), the most common types of cancer are the brain and other central nervous system tumours and lymphomas, followed by leukemias, gonadal (testicular and ovarian) germ cell tumours, thyroid cancer, and epithelial cancers such as nasopharyngeal and salivary glandular cancers.


Common adult cancers (lung, breast, colon, and others) rarely occur in children or adolescents. Childhood cancers tend to be more aggressive than adult cancers.


Childhood cancers are rare, and hence only specially-trained doctors are equipped with knowledge and experience to properly treat them. In fact, children with tumours/cancers need to be treated by a multidisciplinary team of paediatric oncology specialists. This multidisciplinary strategy ensures that children receive the treatment, supportive care, and rehabilitation therapies that will give them the best chance at not only survival but a good quality of life.

Leukaemia

Leukaemia is the most common cancer in children and teens, accounting for almost 1 out of 3 cancers. Most childhood leukaemia’s are acute lymphocytic leukaemia (ALL). Most of the remaining cases are acute myeloid leukaemia (AML). Chronic leukaemia’s are rare in children.


Leukaemia is cancer that arises from blood cells. Based on the blood cell of origin, they are broadly classified into lymphoid leukaemia and myeloid leukaemia. The treatment of leukaemia’s consists of systemic therapy with or without bone marrow transplantation.


  • Acute lymphoid Leukaemia
  • Acute myeloid Leukaemia
Brain and spinal cord tumours

Brain and spinal cord tumours constitute a major percentage of childhood solid tumours. They are classified based on location, cell of origin and other molecular factors. The most common tumours are as follows:


  • Gliomas
  • WHO classification of gliomas
  • Medulloblastoma
  • Atypical TeratoidRhabdoidTumours
  • Primitive neuroectodermaltumours and pineoblastomas
  • Craniopharyngioma
  • Ependymomas
  • Germ Cell Tumours - Non-germinomatous and germinomas

Pituitary adenomas


  • Secretory
  • Non-secretory

  • Neurocytomas
  • Choroid plexus tumours
  • Meningioma
  • Schwannoma
  • Spinal tumours

Patients with brain tumours are treated with a combination of surgery, radiation therapy and chemotherapy. Several recent advances such as minimal invasive surgeries, high precision radiotherapy techniques such as stereotactic radiation therapy and Proton beam therapy as well as targeted therapies have significantly impacted the management of these tumours.

Neuroblastoma

Neuroblastomas are tumours that arise from neuroblasts or “early nerve cells” arising from the sympathetic nervous system. Most of these tumours arise in the abdomen, half of which arise from the adrenal glands which are glands located just above the kidneys. It may also arise in the chest, neck, or the pelvic region. The patients undergo genetic and molecular testing apart from the usual test such as biopsy, imaging and bone marrow examination. The treatment consists of a combination of systemic therapies, surgery and radiation therapy. Rarely some of the tumours stop growing and mature on their own to form normal nerve cells. Several recent advances have changed the management of these tumours for good.

Wilm’s tumour

Wilm’s tumour or nephroblastoma is a rare kidney tumour that affects younger children younger than 5 years. It commonly affects the kidney on one side but occasionally involves both kidneys. Occasionally certain genetic alterations that are passed from parent to children could be associated with Wilm’s tumours. Wilm’s tumours are also associated with other abnormalities at birth including aniridia (lack of coloured portion of the eye) and hemihypertrophy (significantly larger one half of the body) or as part of certain genetic syndromes such as Denys-Drash syndrome and Beckwith-Weidman syndrome.


Recent advances in the understanding of the biology of these tumours and in the management of these tumours have led to significantly better clinical outcomes.

Rhabdomyosarcoma

Rhabdomyosarcoma develops in the muscles and usually presents with pain or swelling. The embryonal form, which is more common in children, has a better prognosis than its counterpart, alveolar rhabdomyosarcoma. Also, we do perform genetic mutational analysis for embryonal form, which is taken into account during treatment. Rhabdomyosarcoma treatment encompasses a combination of chemotherapy, radiation therapy/proton therapy and surgery, in a systematic protocol. In a high volume centre, the outcomes are likely to be better compared to centres that do not treat many children with this diagnosis.

Retinoblastoma

Retinoblastoma is a cancer of the retina, a light-sensitive layer of tissue in the eye. Among children, it is the most common malignant tumour that starts in the eye. Still, it is very rare and accounts for 3% of childhood tumours. It can be hereditary (25-40%) and sporadic (60-75%). Treatment includes intra-arterial chemotherapy, intravitreal chemotherapy, systemic chemotherapy, laser therapy, cryotherapy, enucleation, and radiation/proton therapy.

Ewing’s sarcoma

Ewing’s sarcoma or Ewing’s group of tumours are seen in bones and soft tissues. They are usually seen in the pelvis, chest, or legs, particularly the long bones. Rarely, tumours grow in the skull or flat bones in the trunk. Treatment for these tumours includes surgery, including limb-sparing surgery, chemotherapy and radiation/proton beam therapy. Prognosis of these group of tumours are usually good, and as said, earlier the stage is better.

Osteosarcoma

Osteosarcoma is the most common type of bone cancer in children, teens, and young adults. Usually they present as tumour near the knees. Treatment for these tumours include surgery, including limb-sparing surgery, chemotherapy and radiation/proton beam therapy. Treatment will be tailored according to individual needs.

Lymphomas
Other Bone sarcomas
Paediatric Nasopharyngeal carcinomas
What causes cancer in children?

Most childhood cancers do not have a known cause. Less than one-tenth of all cancers in childhood are caused by a mutation which can be passed from one generation to the next. For example, less than half of children affected with retinoblastoma, a tumour of the eye that is seen in children, inherited a mutation in from either parent. Inherited mutations associated with certain familial syndromes, such as Li-Fraumeni syndrome, von Hippel-Lindau syndrome, Beckwith-Weideman syndrome, Fanconianemia syndrome, and Noonan syndrome also increase the risk of childhood cancer. Occasionally Leukaemia’s are associated with Down syndrome.


Most cancers in children, like those in adults, are thought to develop as a result of mutations in genes that lead to uncontrolled cell growth and eventually cancer.


The factors which have an association with such mutations are ionizing radiation directly or to the parents (especially to the mother during the pregnancy), maternal consumption of curated meats, exposure to tobacco smoke, pesticides and certain chemicals such as solvents, petrochemical compounds, and excessive outdoor pollution.

What is the prognosis of childhood cancers?

The overall prognosis of children and adolescents with cancer has improved significantly over the last few decades. The improvement has been dramatic in a few cancers such as acute lymphoblastic leukaemia where the 5-year survival rate has improved from 57% in the mid-’70s to 92% in 2012. Similarly, in Non-Hodgkin’s lymphoma, the 5 years’ survival rates have improved from 43% to 91% during the same period. Because of these dramatic improvements, brain tumours are now the leading cause of mortality in children. Tumours such as Wilm’stumours, neuroblastomas, rhabdomyosarcomas and certain bone sarcomas have seen a moderate improvement in prognosis. Children with aggressive tumours such as diffuse intrinsic pontine glioma continue to remain a matter of concern.


According to a recent publication it is estimated that 4,16,500 children and adolescents between the ages of 0 to 19 were diagnosed with cancer and 142,300 have died of this disease in the year 2017 (GBD 2017, Childhood Cancer Collaboration). Between 1999 and 2014, the cancer death rate dropped the most for 1- to-4-year-olds (a 26% drop), followed by that for 15- to 19-year-olds (a 22% drop), 10- to 14-year-olds (a 19% drop), and 5- to 9-year-olds (a 14% drop).

Where does lymphoma usually start?

Lymphoma usually starts in the lymphatic system, which comprises bone marrow, lymph nodes, spleen, abdomen, chest, intestines and skin. As lymphatic tissues are usually present everywhere, they can practically arise from anywhere in the body.

What are the odds of a child getting cancer?

The odds of a child getting cancer as per the latest reports are about 1 in 330. In India alone, 50000 children are affected by cancer every year, but the numbers can be higher, as close as 75000.

Why APCC for Paediatric CMT?

Apollo Proton Cancer Centre provides comprehensive, cutting edge care for children, and adolescents affected with paediatric tumours/cancers thereby resulting in excellent clinical outcomes. All patients are handled by paediatric specialists including paediatric hemato-oncologists, paediatric surgeons, paediatric radiation oncologists, paediatric nurses, paediatric counsellors and other specialists such as endocrinologists, ophthalmologists, infectious disease specialists, intensivists, orthopaedic surgeons, psychologists, physiotherapists, etc.


APCC also is equipped with South Asia and the Middle East’s first and only proton beam therapy facility. The centre has dedicated areas which have been made child friendly, so as to enable safe and seamless treatments. The centre also has a dedicated paediatric anaesthesia team for safe delivery of short sedation to enable imaging, radiation/proton therapy and surgeries.


Every child’s clinical condition is discussed in the multidisciplinary tumour board consisting of all the relevant specialists. A decision regarding the best combination of treatment to achieve the best possible outcomes is taken in an unbiased way and communicated with parents. MDT approach has been shown to improve decision making as well as improve outcomes in challenging clinical scenarios. MDT’s also take into account the patient/family’s preferences in the overall treatment decisions.

Symptoms

 

In leukaemia’s and lymphomas, usual symptoms are
  • Fatigue
  • Fever
  • Weight loss
  • Bleeding
  • Bone and joint pains
In brain tumours, it usually involves
  • Headache
  • Vomiting
  • Dizziness
  • Seizures
  • Imbalance
  • Gait Disturbances
  • Eye Incoordination etc
Tumours like neuroblastoma have
  • High blood pressure
  • Impaired ability to walk
  • Pain
  • Diarrhoea
Whereas Wilms tumours usually involve
  • Swelling of the abdomen
  • Fever
  • Nausea
  • Poor appetite

Unusual symptoms and signs should start with a visit to the clinician for further evaluation and management.

Children’s first symptom of leukaemia

Most of the time, the presenting symptoms are non-specific but may present with fatigue, fever, weight loss, bleeding, bone and joint pains.

Signs of lymphoma in children

Common symptoms of lymphoma include lymphadenopathy, mediastinal mass and systemic symptoms like fever, anorexia, weight loss, night sweats. Sometimes, enlargement of the liver and spleen can also occur, and very rarely autoimmune haemolyticanaemias i.e. reduction in the red blood cell, white blood cell and platelet counts.

Risk Factors

 

Most children have no known risk factors but some of the well-known risk factors are as follows:


  • Certain conditions or syndromes such as Down syndrome and others where the risk of development of cancer is high (10-20 times increased risk in Down syndrome).
  • Certain genetic mutations: Tumours such as retinoblastoma, Wilm’stumours can be associated with inherited genetic mutations. These are found through genetic testing in families associated with medical problems associated with these genes.
  • Changes during fetal stage: Certain tumours such as Wilm’stumours, neuroblastoma and retinoblastoma develop during this phase due to certain genetic alterations.
  • Infective causes: Viruses such as Epstein Barr Virus (EBV) (is associated with childhood malignancies such as nasopharyngeal carcinoma, certain lymphomas) and HIV are associated with certain childhood malignancies.
  • Radiation exposure: Radon gas exposure through several natural sources and medical radiation exposure can increase the risk of cancers.
  • The previous diagnosis of cancer or treatment of cancer is associated with an increased risk of cancer.
What increases the risk of childhood cancers?

Compared to adult malignancies, environmental factors have not been found to play a major role in childhood cancers. Some of the common factors that increase the risk of childhood cancers are radiation exposure, medical conditions like Down’s syndrome, genetic abnormalities, developmental abnormalities in the womb, and associated infections.

Genetic and non-genetic risk factors for childhood cancers?

Genetic factors include acquired or inherited mutations causing DNA damage in children. Some of the genetic factors like abnormalities in the Retinoblastoma gene, p53 gene, leads to different malignancies later in childhood. Non-genetic factors include exposure to infections like Epstein Barr virus, problems associated with development while inside the womb, previous radiation exposure, history of chemotherapy increases the risk of childhood cancers.

Diagnosis

 

How are childhood cancers diagnosed?

Most of the childhood cancers symptoms are often noted by parents or relatives. Unusual body swelling or lump, pallor, bruises, unexplained fever and weight loss, headache, vomiting, seizures, eye or visual changes, limping, warrants a visit to the clinician.

How do you diagnose leukaemia in children?

Leukaemia’s are usually suspected when a child presents with persistent fever, weight loss, bleeding/bruising, bone pain, hepatosplenomegaly and/or lymphadenopathy. Some of the common investigations done are complete blood panel, peripheral smear, bone marrow examination, and flow cytometry/immunochemistry are done to confirm the diagnosis of leukaemia.

Treatment

 

Various treatment options and surgery

Treatment of childhood cancers, depending on the type of cancer and the site of disease.


Common treatment options for childhood cancers include Systemic therapy (Chemotherapy/Targeted Therapy), Surgery (Complete or partial removal of tumour) and Radiation therapy/proton therapy (Alone or in combination with systemic therapy/Surgery).

What is the survival rate?

The survival rate depends on the type and stage of the disease. Recently, outcomes of treatment in certain tumours can be predicted by using molecular information from the tumours as a tool. Certain childhood tumours when diagnosed early the cure rates can be as high as 95%, and therefore survival in those children is similar to that of a normal population.

How long is chemotherapy for childhood cancers?

The duration of chemotherapy also depends on the stage and type of tumour. The duration depending on the type of tumour ranges between 3 months to 1 year or more in some children. The chemotherapy is administered usually as injections or tablets over a few days to weeks followed by a gap before the next dose.

Preparing your child for medical procedures?

Some parents think they should not tell a child about an upcoming procedure, especially if it may hurt. But children need clear and honest information. If you tell your child that a procedure will not hurt, and when it does, the child may be surprised and confused. Your child may then expect all procedures to hurt and will not believe you if you say otherwise.


Many children can cope with pain, loud machines, or uncomfortable positions if they know when it will happen and how long it will last. Learning this information ahead of time can help your child share what he or she needs to stay calm during the unpleasant experience. You may want to explain to your child that unpleasant things have to be done to help him or her get better. But remind your child that this procedure is not happening because he or she did anything wrong. A social worker, nurse, child life specialist, or another member of the health care team can help you find the right words to explain the medical procedures to your child.

Childhood cancer treatment side effects?

Side-effects depend on the type and nature of the treatment. Most of the side effects are temporary. Children recover from them a few months after treatment. However, there are some side-effects that can be long-term. With recent advances in the field of medicine and by using sophisticated technology such as Proton therapy, targeted therapy, minimally invasive surgical techniques, etc the long-term side-effects can be minimized.

Childhood cancer treatment side effects?

Side-effects depend on the type and nature of the treatment. Most of the side effects are temporary. Children recover from them a few months after treatment. However, there are some side-effects that can be long-term. With recent advances in the field of medicine and by using sophisticated technology such as Proton therapy, targeted therapy, minimally invasive surgical techniques, etc the long-term side-effects can be minimized.

Ways to prevent childhood cancer?

Unlike many cancers of adults, lifestyle-related risk factors (such as smoking) don't play much of a role in a child’s risk of getting cancer. A few environmental factors, such as radiation exposure, have been linked with an increased risk of some childhood cancers. But in some cases, exposure to radiation might be unavoidable, such as if the child needs radiation therapy to treat another cancer.

Life and health after childhood cancer?

During cancer treatment, most families are mainly concerned about the daily aspects of getting through treatment and beating cancer.


It is certainly normal for families to want to put cancer and its treatment behind them and to get back to a life that doesn’t revolve around cancer. But it’s important to realize that close follow-up care is a central part of this process that offers children the best chance for recovery and long-term survival.


Once treatment is finished, the health care team will set up a follow-up schedule. For many years after treatment, children must have regular follow-up exams with the cancer care team. As time goes by, the risk of cancer coming back goes down. Doctor visits might be needed less often, but they are still important because some side effects of treatment might not show up until years later.


Because of major advances in treatment, more children treated for cancer now survive into adulthood. But treatments might affect children’s health later in life, so watching for health effects as they get older has become more of a concern in recent years. The earlier any problems are recognized, the more likely it is they can be treated effectively.

Does surviving cancer shorten life expectancy?

It is true that surviving cancer may shorten life expectancy because of the late side-effects related to treatment. However, these effects can be mitigated by following a lifestyle. Evolving treatment approaches are projected to be associated with improved life expectancy after treatment for paediatric cancer.

Side-effects

Side-effects depend on the type and nature of the treatment. Most of the side effects are temporary. Children recover from them a few months after treatment. However, there are some side-effects that can be long-term. With recent advances in the field of medicine and by using sophisticated technology such as Proton therapy, targeted therapy, minimally invasive surgical techniques, etc the long-term side-effects can be minimized.

Does childhood cancer come back after treatment?

Depending on the stage and type of cancer, there is a risk of cancer coming back later. However, in certain childhood tumours when diagnosed early the cure rates can be as high as 95%. Once treatment is finished, the health care team will set up a follow-up schedule. For many years after treatment, it is very important that children have regular follow-up exams with the cancer care team. As time goes by, the risk of cancer coming back goes down.

Psychological effects of childhood cancer?

Cancer and the treatment of it can have serious social and emotional consequences for the child. Children with cancer and survivors of childhood cancer may experience severe anxiety, inhibited and behavioral problems, excessive somatic complaints, intense stress, post-traumatic stress disorder (PTSD), academic difficulties and surrounding frustration, peer relationship difficulties, and worries about the future concerning with the career and relationships. Family members of a child with cancer often suffer various forms of distress about the child’s illness.


Early signs of emotional distress and symptoms of posttraumatic stress require early assessment and intervention to minimize the negative impact on the child and his or her family. APCC has an excellent psychosocial support team constituting psychiatrists, psychologists, counsellors specializing in managing paediatric, teenage and young adult cancer patients.


Late effects of childhood cancer treatment. The treatment of cancer may cause health problems for childhood cancer survivors months or years after successful treatment has ended. Cancer treatments may affect the body's organs, tissues, or bones and may cause health problems later in life. These health problems are called late effects.


Doctors at APCC are studying the late effects caused by cancer treatment. They are working to improve cancer treatments and stop or lessen late effects. While most late effects are not life-threatening, they may cause problems that affect health and quality of life. The cutting edge pencil beam scanning proton therapy used at APCC has shown promising results in preventing/minimizing these late effects and helping children maintain a good quality of life after cancer treatment.

Life beyond paediatric cancer

Children treated for cancer miss out on a lot of normal social things. They miss school and are often isolated. Social developmental milestones can be delayed. Receiving cancer treatment as a growing child can have long term impacts on physical and emotional health – especially a developing child. Adolescents who are treated often lose independence at a time that they would normally be establishing independence.


In addition to the many physical late effects that may develop in survivors of childhood cancer, there are also a host of psychosocial issues that may emerge. Long-term educational, social, behavioural and vocational difficulties are all common.


APCC has a comprehensive program “AALAP” for providing comprehensive care to all children, teenagers and young adults with cancer.

Clinical trials

APCC has an active clinical research unit whereby our doctors are in a constant endeavour to bring out innovative treatment strategies to minimize side effects if any due to cancer treatment and help children in marinating an active life even after treatment.

Research in paediatric oncology

In recent years research related to childhood cancers has made tremendous progress. APCC’s paediatric CMT has been actively involved in studying the effect of various treatments in childhood cancer patients.

Winning over Cancer with Apollo Proton Cancer Centre

The growing burden of cancer across the globe tells an ominous tale. To counter this growing threat, Apollo Hospitals introduces Proton Beam therapy with focused cancer management teams that will provide optimal solutions like never before. We believe it is critical to redefine our purpose, to reboot our commitment on the single-minded focus to win over cancer. APCC stands as a ray of hope for millions of patients as they join us in the battle to win over cancer.