Modern Pediatric Oncology Practice and the Key Elements of Cancer Cure

With timely diagnosis and optimal treatment, eight out of 10 children with cancer get cured. For certain cancers like Hodgkin lymphomas, germ cell tumours and Wilms tumour survival is >95% while for others like acute lymphoblastic leukemia and Burkitt lymphoma  it now approached 90%. Curing a child of cancer gives the child and the nation 60-70 years of a good productive life. The following are the key elements of providing pediatric oncology care in the 21st centrury and have contributed to the improved survival in children with cancer.

1. Timely and Accurate Diagnosis

Suspecting a malignancy, investigating for the same and referring in a timely manner are the first critical steps towards long term cure.Figure 1A and 1B is a diagrammatic representation of when a paediatrician or a physician should consider evaluation for an underlying malignancy.

Figure 1 A and 1BEarly Warning Signs of Childhood Cancer taken from the Awareness Media Campaign of UICC and SIOP

The first few basic investigations in a suspected leukaemia include a bone marrow evaluation. An extensive immunophenotyping (flowcytometry) apart from morphological examination and cytochemical staining of bone marrow smears to discern the type of leukaemia (B or T phenotype Acute lymphoid leukaemia (ALL), Acute myeloid leukemia (AML) or biphenotypic leukemia). Further advancement in evaluation of bone marrow samples in form of cytogenetics and PCR based molecular workup for certain specific mutations allows to further ascertain various prognostic factors specifically in haematological malignancies.

Children who present with mass lesions should undergo a definitive biopsy after appropriate radiological imaging. Apart from usual imaging, PET CT has been emerging as an essential tool in determining the extent of disease, response to treatment and recurrence in many pediatric malignancies. Use of immunohistochemical markers (IHC) help in determining the exact characteristic and differentiating the various pediatric round blue cell tumors. FNAC is insufficient in most of the case and is therefore, discouraged as it leads to unnecessary delay in diagnosis and institution of definitive treatment. Biopsy of the mass lesion should be performed at the centre expertise in pediatric oncology care so that the biopsy scar can be included in the definitive treatment. It has been commonly observed that unplanned biopsies of specifically limb lesions render the otherwise salvageable limb to undergo amputation. Like the molecular analysis of bone marrow sample in haematological diseases, the tissue biopsy block is also subjected to various molecular tests like N-MYC amplification in neuroblastoma which upstages a disease and mandates an aggressive treatment.

2. Risk Stratified Protocol Based Treatment Provided by a Multidisciplinary Team

Risk stratified treatment based on clinical and biological parameters including early response is the essence of pediatric cancer treatment in the modern era. Various cytogenetic and molecular analysis as discussed above along with other patient variables help the oncologist to determine the stage of disease or classify it into low, intermediate or high risk disease category. Response to first few chemotherapy cycles is the most important predictor of long term chance of cure in a majority of diseases. This ameliorates the toxic effects in good risk disease and intensifies treatment in high risk disease.

Mutidisciplinary care including the radiation oncologists, onco-surgeons, pathologists, radiologists incuding nuclear medicine physicians play a pivotal role in care of these children. This allows us to provide scientifically proven protocol based treatment that is accepted worldwide. Strict adherence to the protocol is essential to get the desired results. Many of these protocols are created in the context of multicentre prospective clinical trials. The Indian Pediatric Oncology Group (InPOG) is spearheading efforts in this direction. At Max group of hospitals, we currently have ongoing InPOG trials in childhood ALL and Hodgkin disease.

3. Supportive care

A major advancement in cancer cure in last few decades is attributed for to an improved supportive care. This includes

1. Improved venous access: Venous access devices like PICC line, tunnelled Hickman or Brioviac type catheters and implantable ports allow easy chemotherapeutic drug delivery with decreased risk of extravasation, prolonged intravenous fluids and blood products administration and obviates the need of repeated needle pricks for various blood tests.
2. Safe transfusion practice: Disease and its treatment both may cause myelosuppression. Availability of NAT tested, leukodepleted and irradiated blood component support is an essence of cancer treatment armamentarium.
3. Infection control: Children with cancer are rendered immunocompromised due to disease and treatment. They are susceptible to their endogenous flora and drug resistant organisms. Adequate hand hygiene, aseptic care of central venous access devices, environmental and personal hygiene, hospital antibiotic policy contribute towards lesser neutropenic complications.

Supportive care cannot be delivered without the efforts and dedication of skilled nursing staff as well as other essential pediatric sub-specialities like intensive care, cardiology and nephrology whose support is critical to tide over the critical phases during intensive oncology treatment. Last but not the least is the help and constant support of the NGOs, who not just help in arranging funds and facilities for those who cannot afford it but also provide psychological support to the adolescents, parents. They bridge the gap between the physician and parents, form parent support groups and motivate the newly diagnosed family to continue treatment and lessen the abandonment rate.

4. Continued Care of Survivors

Preventing, monitoring and treating late effects of cancer and its treatment in childhood cancer survivors is essential. Time has come to look beyond cure. As more and more children achieve overall survival rate of more than 80%, it becomes important to give them a good quality of life.Risk adapted treatment is an important step towards reducing treatment for good risk diseases and hence, in ameliorating the future toxic effects of cancer therapy. Also a long term follow upto identify the signs and symptoms of late organ toxicity become equally important and empower us to act efficiently ensuring a good quality of life. The InPOG-LE-16-01 study is a step towards achieving the same where childhood cancer patients are enrolled onto a survivor registry at end of treatment and monitored subsequently.

Essential role and responsibility of a Pediatrician

Pediatricians play the most important role in this complete endeavour. When a primary care pediatricican has a high index of suspicion for a childhood cancer, the child is likely to be referred to the correct facility. They may act as a primary care-giver for the supportive care when the child is unable to access the oncology center due to logistics. They play an eminent role in encouraging the family in completing desired therapy and enhancing potential long term cure.Immunisations post therapy completion and follow up can be taken up by the primary pediatrician and hence form an important link between the oncologist and family.