Paediatric Cancers

Paediatric Cancers

Paediatric Cancers

Any abnormal growth of the cells of the body that might invade the normal organs and can spread to and grow at distant sites in the body is known as a cancer. Cancer can arise in any part of the body and at any age. The cancers in children are different from those in adults in their behaviour and require different treatment approaches. In India over 70,000-80,000 children are diagnosed with cancer every year. Out of them only about 5% have a genetic predisposition or family history. The cause of the rest remains unknown.

The past decades have seen a tremendous improvement in the outlook of children living with cancer. The survival in high grade/stage tumours has increased to more than 70 % and that for lower stage has increased to greater than 90%. Some tumours, if detected at the right time have become completely curable.

Does my child have cancer?

The symptoms of childhood cancer are extremely variable depending on the organ of origin and the extent of spread. As an example tumours of the brain may cause minor behaviour changes, liver tumours might present as jaundice and tumours of the lymphatic system might just present with unexplained fever.

There are how-ever certain danger signs that should prompt immediate consultation with the doctor. These include:-

  1. A lump or swelling anywhere in the body
  2. Unexplained fever, loss of weight , loss of appetite
  3. Child appears pale, easy bruising/bleeding, body aches and easy fractures
  4. White spot on the eye or recently developed eye problems
  5. Unusual behaviour or movements

What is the role of a pediatric surgeon?

The pediatric surgeon is usually a cornerstone of treatment of pediatric tumours. They provide surgical support and often act as liason between the various other doctors who are involved in the treatment of the child. Most children with cancer will need surgery in some form or the other during the course of the treatment. The common surgeries that are performed are:-

  1. Biopsy
  2. Tumour excision
  3. Chemo Port placement
  4. Surgery for obstructions
  5. Management of complication of other treatment procedures.

What are the other treatment modalities available?

Based on the type of cancer and its stage a variety of treatment approaches are available apart from surgery.

  1. Chemotherapy:- This consists of drugs that are given to stop the cancer cells from dividing. These are usually given by a pediatric oncologist, who decides the optimal regimen based on the tumour type, stage and the side effects of each drug.
  2. Radiotherapy:- This involves the delivery of high energy radiation that is directed at the tumour cells in order to kill them. The radiation is delivered with the help of special machines that help the radiotherapist to control the dose and minimise the side effects.
  3. Bone marrow transplant:- At times the cancer itself or due to its treatment the bone marrow of the child might get destroyed. The child is then unable to produce blood cells adequately. Such children need bone marrow transplantation to replenish the damaged marrow.
  4. Newer therapies:- cancer treatment is an area of active research and constantly there is the discovery of newer drugs and invention of better procedures to fight the disease. These treatments are specific to the cancer type and are usually offered in an individualised manner.

Wilms tumour

It is a common kidney tumour that is usually seen in children less than 5 years of age. It usually involves one kidney though rarely involvement of both sides is seen. Typically presents as a lump in the abdomen of a previously healthy child. Blood in urine and high blood pressure may also be seen. It is known to be associated with certain syndromes like WAGR, Denys-Drash syndrome and Beckwith Weideman syndrome. The diagnosis of Wilms’ tumour is usually made on the basis of physical examination and CT/ MRI scan.

Most children then need to undergo surgical excision of the tumour followed by chemotherapy and radiation as a part of treatment. Sometimes the tumour may be very advanced. In this situation usually a biopsy is done to confirm the diagnosis followed by chemotherapy. The surgical removal is then planned for once the tumour reduces in size.

The outcome of children living with Wilms tumour is very good with survival of 90% depending on the stage and histology of the tumour. Early diagnosis and comprehensive care is needed to ensure the best outcomes in these children.

Neuroblastoma

It is a solid cancerous tumour, arising from the nerve cells, seen in infants and young children. Most commonly seen in children less than 5 year old and is rare above the age of 10. It typically occurs in the adrenal glands, but can occur anywhere in the chest and abdomen of the child. It is the second most common pediatric cancer accounting for about 8-10% of all childhood cancers. Neuroblastomas are typically seen as painless masses that the parents notice while bathing / cleaning the child. It can also present with weakness of the limbs or with bluish painless nodules on skin. The diagnosis of neuroblastoma is made based on blood tests and various imaging tests like CT , PET scan etc. The treatment of the neuroblastoma is multimodal and is decided based on the risk stratification of the child i.e low, intermediate or high risk. Chemotherapy , surgery, radiation and bone marrow transplantation is tailored for the individual patient. The outcomes of the children ranges from being excellent for the low risk category of patients ( ~98% survival) to very guarded for those in the high risk group (40-50% survival).

Rhabdomyosarcoma

It is a rare cancerous growth that arises from the connective tissues ( tissues which connect, support and surround organs and other body structures) of the child. It most commonly affects children and young adults between ages of 2-25 years. Majority of the cases are seen in less than 10 year olds. Rhabdomyosarcomas are usually asymptomatic to start with and may cause problems to the child once they are large and start affecting the organs. The presentation is very varied and depends on the organ system that is involved. Typically diagnosed due to non resolving symptoms and timely evaluation by a pediatric surgeon helps catching them early. Once the diagnosis of rhabdomyosarcoma is made a multi-disciplinary team along with a pediatric surgeon will determine the stage of the disease and the most effective treatment. The management might include surgery, chemotherapy and radiation in various combinations. The management of rhabdomyosarcomas might result in loss of organs or their parts, therefore these children might need further reconstructive surgeries to enable normal life. The prognosis of rhabdomyosarcoma is extremely variable and depends on the individual case. Typically the survival rate of patients with no metastases is 60-80% while those that have distant spread, have about 30% survival.

Sacrococcygeal teratoma

It is a tumour that occurs in babies at the base of the tailbone. It is characterised by the presence of different types of tissues inside the tumour. These tumours may be detected during pregnancy and need treatment after birth. These are the most common tumours seen in newborns and typically seen as lump at the base of the spine of the baby. Sometimes the presence of a large SCT might result in a cesarean delivery and preterm child. A pediatric surgeon needs to be consulted immediately after birth in these patients. When present a sacrococcygeal teratoma needs to be evaluated in the form of blood tests and MRI to see the extent of the tumour. Once the evaluation is done the child needs to undergo surgical removal of the tumour, following which the histopathological examination is done to confirm the diagnosis. During the surgical removal of the tumour it is important to properly reconstruct the muscles and the ligaments of the region for optimal outcomes. Further treatment in the form of chemotherapy may be needed if the tumour is found to be cancerous. Early diagnosis with excision is curative in patients with sacrococcygeal teratomas. Although cancerous changes may be seen in 10-20% of the patients the outcomes are very good.

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