Dr Lucky Gupta
Cancer is a genetic disease which is caused by mutations or alterations in the genetic code of the cell by various agents like carcinogenic chemicals, radiation, viruses or may be heredity (5%). International Childhood Cancer Day is celebrated on 15th February each year. In children, injuries are the first cause of mortality and cancer is the second most common cause of death. In India, the incidence of childhood cancer is approximately between 1.5-4.8%.
Clinical Presentation
In children, the clinical presentation of the cancer can be seen either in the form of classical signs and symptoms, which directly help in diagnosis, like in childhood leukemia, or atypical presentation, where the diagnosis is difficult and a sense of suspicion has a great role. The nonspecific symptoms are seen in the form of irritability, lethargy, weight loss, anemia, feeding difficulties etc. The common tumors seen in childhood are leukemias, neuroblastoma, wilm’s tumor, liver tumors, rhabdomyosarcoma and germ cell tumors. Sign and symptomatology also vary depending upon the primary or metastatic site involvement. If there is spine involvement there may be weakness or paralysis of the area supplied, if extensive bone marrow involvement, then petechial hemorrhages, if lung metastasis, then respiratory symptoms will be seen. Sometimes, other congenital anomalies like Down’s syndrome, common cloaca, limb hypoplasia, spina bifida, etc., are also seen in these patients.
Diagnostic investigations
Investigations mainly depend upon the affected anatomic site and suspected pathology. With the development and improvement of the imaging modalities like prenatal ultrasonography (US) and magnetic resonance imaging (MRI), a significant improvement in diagnosis and management is seen in the form of fetal outcome. Similarly, contrast-enhanced computed tomography (CECT) is helpful in providing excellent images in most neoplasms except if there is intraspinal involvement where MRI has led. The use of innovations in the image – guided percutaneous biopsy techniques or diagnostic tumor core biopsies has really helped in improvement in making diagnosis in contrary to open biopsy techniques. Also, positron emission tomography (PET) is also used in the management of selected children with malignancy. The role of Cytogenetics like N-myc amplification, which is a specific molecular marker, cannot be ignored in the diagnosis and risk stratification of neuroblastoma patients.
Treatment
Treatment of affected children especially infants is extremely challenging due to age, the immature immune system, the biological behavior or sometimes the unpredictable course of the tumor, like spontaneously involution as seen in congenital neuroblastoma or progression to a fatal outcome as seen in sacrococcygeal teratoma as if untreated in infancy can become malignant. Generally, the patient requires multimodal treatment especially if in advanced stage of cancer.
Surgical Treatment
Surgery is a mainstay treatment in most of the children having solid tumors. For effectiveness of the treatment, the physiologic and metabolic needs of the children especially neonate, the timing of the surgical procedure and the surgical strategies should be considered. As the children are in growing phase, so the surgical procedures will definitely affect the subsequent growth and development especially when extensive surgeries or resection of unaffected tissues integral for normal structure and function will be performed. So, here the important principle is the adaptation of the multimodal strategies in order to preserve the function and structure and this can be gained by adjuvant chemotherapy for the tumor which is initially unresectable or involves important structures.
Radiotherapy
Malignant tumors in childhood are mostly radiosensitive and especially the advanced-stage tumors. There is direct relationship with the radiation dose of radiation therapy and its adverse effects and inverse relationship between the sensitivity and detrimental effects and the child’s age. The involvement of various body systems and organs will affect the growth of the child accordingly and these affects can be seen in the form of delay in cognitive development, renal and hepatic insufficiency, scoliosis, learning disabilities along with the second malignancies like leukemias, breast carcinomas etc.
Chemotherapy. Various drug interactions, metabolism, clearance, and toxicity all are important while considering chemotherapy. Complications like neurotoxicity hepatic toxicity ototoxicity with vincristine, actinomycin D, and cisplatin are known. Drug dosage adjustments along with the innovations in drug delivery and monitoring has made significant improvement in reducing their side effects.
Also, the use of novel therapeutics like targeted agents (antibodies, cellular therapies, kinase inhibitors) and stem cell transplantation has significantly led to the improvement of childhood cancer management along with the programs for cancer predisposition, testing and genetic counseling both for patients with cancer and their family members.
(The writer is Assistant Professor, Department of Pediatric Surgery,
AIIMS, Jammu).
