CANCER IN CHILDREN IS CURABLE: IMPLICATIONS FOR THE PEDIATRICIAN
Dr. B. R. Agarwal*
Head, Dept of Pediatric Hematology-Oncology, B.J.Wadia Hospital for Children, Parel, Mumbai 400012.*
Keypoints
  • The concept that cancer is incurable needs prompt revision
  • A specialized pediatric tertiary care center offering a multidisciplinary team to manage the pediatric oncology patients is the best bet for achieving desired results
  • Educating and encouraging the pediatrician to take part in the shared care of the pediatric oncology patients is a realistic strategy to improve childhood cancer survival
  • The pediatrician is an important link between the patient, his family on one end and the pediatric oncologist on the other end

Not only the lay public, but also even many within the medical fraternity in India believe that cancer is potentially incurable. This concept needs prompt revision. Reliable data from advanced nations have proven that cancer is often curable in children e.g., statistics from USA have shown that in children with cancer under 15 years of age and diagnosed between the years 1990 to 1993, the 5 year survival rate is 68% (which means cure!). Even though we are a largely illiterate country with limited resources, still grappling with infectious diseases and malnutrition; attempts must be made to reach a more modified goal as regards cancer management. This can be achieved by developing an optimistic attitude and realistic strategies. These include, initiating a nationwide programme to create awareness about cancer in the people; educating and encouraging the pediatrician to take part in the shared care of the pediatric oncology patients; and devising a framework for organized Pediatric Oncology service at various centers in the whole of India. This can be done based on data that we and others from advanced industrialized countries have gathered.

A few facts provide ideas on the need to focus on pediatric oncology care:
  • The 5-year survival rate in children with cancer is nearly 70%: USA reports a cure rate of 70% in children with cancer below the age of 15 years. In decreasing order of frequency childhood cancers are Acute Lymphoblastic Leukemia (ALL), Brain astrocytoma, Neuroblastoma, Non-Hodgkin's Lymphoma (NHL), Wilms' tumor (WT), Hodgkin's Disease (HD). Peripheral Neuroectodermal tumor (PNET), Acute myelogenous leukemia (AML), Rhadbo-myosarcoma (RMS), Retinoblastoma, Osteosarcoma, Ewing's sarcoma and other less common ones. A break-up of the probability of cure rate in various malignancies, expressed as EFS (Event-free survival) or RFS (Relapse-free survival) or DFS (Disease-free survival) or PFS (progression-free survival) is outlined in Table 1.1. With such magnificent results, the effort to treat these cases is worthwhile and fulfilling.
  • The number of cancer cases rise as countries develop. In the USA, the leading cause of death from disease in children is cancer. As our country develops, we must prepare for a changing pattern in the incidence of various diseases.
  • The type of cancer in children is totally different from those in adults. The response to therapy in children is also different. More details about these differences are described in Chapter 4. In Mumbai, we were surprised to see hepatoblastomas and Juvenile Chronic Myeloid Leukemia (JCML) more frequently than reported in the West.
  • Laboratory backup has helped in diagnosis, management, prognosis and therapy. ALL has been typed immunologically Pre-B ALL in the low-risk group has a complete remission rate of 95% and an event-free probability of 79%. Even in high-risk ALL, the cure rate is 50-70% with present day combination therapy. For NHL, various sources, For W.T., NTWS III, 4 yr RFS, For RMS, IRS II, 5 yr. PFS. In India, we could utilize the latest developments in immunology, cytogenetics, cell and molecular biology and their application in therapy. We also have immediate access to up-to-date worldwide information in these areas.
  • Loss to follow-up of children under treatment for cancer is a common problem in state sponsored hospitals. This accounts for poor outcome. There is an extreme disparity in income, education, nutrition and life-styles between the affluent and the poverty stricken population in India. These will have to be taken into account when planning health care for all children in general and for our purpose in Pediatric Hematology/Oncology.

    Table 1.1: Results of Chemotherapy

    Malignancy

    Type / Stage / Site

    EFS / RFS/*
    DFS/PFS (%)

    Acute Lymphoblastic
    Leukemia (ALL):

    Early Pre-B
    Pre-B
    B-cell
    T-cell

    79
    71
    60
    51

    Neuroblastoma: * Infants < 1 yr.

    A
    B
    C
    D Ds

    100
    100
    88
    79
    73

    * Children

    A
    B
    C
    D

    100
    93
    36
    27

    Wilms' Tumor (WT):

    Stage I
    II
    III
    IV

    89
    87.4
    82
    79

    Hodgkin's Disease:
    (HD)

    CS/PS Stage II & III
    Stage IV

    96
    85

    Rhabdomyosarcoma:
    (RMS)

    Orbit
    Parameningeal
    Other head & neck sites
    Trunk - State I & II Extremity: State I
    II
    III
    IV
    Genitourinary

    78
    70
    78
    71
    95
    67
    58
    33
    79

    Non-Hodgkin's Lymphoma:
    (NHL) - Lymphoblastic

    Stage 1 & 2
    Stage 3 & 4

    67
    79

    Small non-cleaved &
    Burkitt's Lymphoma

    Stage 1 & 2
    Stage 3 & 4

    86
    75


    *From St. Jude Research Hospital, 5 Yr. EFS for ALL, 1984-88, n = 340; for neuroblastoma, 4 yr survival rate, 1979-88, and for H.D., n = 85, Clinical Stage (C.S.), Pathologic stage (PS), EFS.

    Setting up a Pediatric Oncology Centre:
    A specialized pediatric tertiary care center offering a multidisciplinary team to manage the pediatric oncology patients is the best bet for achieving desired results. A list of all centers that treat Pediatric Hematology0Oncology (PHO) cases must be circulated among Pediatricians and during Indian Academy of Pediatrics (IAP) meetings. A local list in the community would help parents when they are confronted with a diagnosis of cancer. Where centers do not exist within a reasonable distance of a community, they need to be established as a Division of Pediatric Hematology-Oncology within the Dept of Pediatrics, in municipal and/or private hospitals. This means persuading the hospital authorities to initiate such a Division of PHO, and persuading the Oncologists trained to treat adult cancer cases and surgeons who also treat cancer cases to refer pediatric cases to a pediatric hematologist/oncologist.

    A proper tertiary pediatric cancer care center should have facilities for:
    • Planned OPD and in-patient units
    • Research, both clinical and laboratory
    • Collaboration with other national and international groups
    • Facilities for educating fellows in Pediatric Hematology/Oncology, medical students, general practitioners, pediatricians, medical technicians, as well as in the community, especially teachers in schools
    • Measures designed for its expansion e.g., Cord Blood Stem cell Transplantation, Blood Banking, Radiotherapy and new laboratory tests as more scientific and clinical information accumulates

    Role of the Pediatrician:
    The pediatrician can collaborate with the oncologists in the shared care of the patients from diagnosis until cure of the child.

    Delay in diagnosis and workup prior to starting therapy pushes a low-risk patient into a high-risk one. Pediatrician can, by physical examination, palpate lumps in the abdomen (Wilms' tumor / Neuroblastoma), watch for CNS signs and symptoms (Brain tumor), and by the liberal use of CBCs (leukemias) detect most of the childhood malignancies. Once a suspicion is entertained and the patient referred to a PHO center, the pediatrician and the Oncologist can map out a program of care together. Whenever feasible and indicated, they can communicate to resolve issues as they arise. Managed care is a local phenomenon, one between the local pediatrician and local oncologist.

    Alert pediatrician can report adverse reactions from chemotherapeutic drugs. We have encountered adverse neurological reactions when intrathecal methotrexate was given at recommended doses in several cases, and on occasion, with severe sequelae. A list of expected reactions to the drugs the child is getting should be made available to the sharing pediatrician.

    One of the major contributions a pediatrician can make is in the area of follow-up, both during and after chemotherapy. "Loss to follow-up" is an exceedingly common occurrence, and with incomplete or intermittent therapy, relapses are inevitable. Pediatricians can encourage and/or coerce the parent to follow through with therapy for potential cure.

    The role of the pediatrician in clinical cancer care should put into place future policy decisions. He should be able to claim a position in the daily care of a cancer patient, and provision for recognition awards should be made. The pediatrician is an important link between the patient, his family and the oncologist. We cannot afford for this link to be weakened by poor knowledge of pediatric oncology as regards the diagnosis and complications of management.
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