DISEASE &
TREATMENTS
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Supratentorial BrainTumors
Brain tumors are second most common neoplasmof childhood after leukaemia, primary brain tumors are responsible for 20% ofall cancers in children, and for 20% of childhood cancer deaths. The yearlyincidence of childhood brain tumors is reported to be 24 per million children lessthan 15 years of age. The neurologic functional outcome and quality of life ofchildren is goal of treatment .
Classification of primary brain tumors inchildren
Brain tumors are classified primarily by theirlocation [supratentorial or infratentorial] and histology. The supratentorialtumors can be sellar, suprasellar, or hemispheric tumors [intraventricular or parenchymal].The most recent World Health organization (WHO) classification schema for tumorsof the CNS is done with criteria of histologictyping of tumors.
Clinical features of childhood brain tumors
Presenting signs and symptoms of primarybrain tumors vary by age and location. Tumors that present in infancy may bemore insidious because of the non-specific nature of clinical features thatarise from a variety of causes, including vomiting, irritability, listlessness,macrocephaly, failure to thrive, and stagnation of development or loss ofdevelopmental milestones. Common signs and symptoms of brain tumors in childreninclude Irritability, Listlessness Vomiting, Headache, Failure to thrive,Macrocephaly, Seizures, Focal neurologic deficits/ hemiparesis, Visual fieldloss/ deficit, Neuroendocrine dysfunction.
Suprasellar Tumors
The sellar and suprasellar region comprisea heterogenous group of lesions that can cause a number of neurological andendocrinological disturbances by virtue of their proximity to the opticapparatus, the hypothalamic-pituitary axis, the diencephalons, and the third ventricle.The histopathology of these lesions varies from benign to highly malignant.Most commonly observed tumors in this region include, germinoma,craniopharyngioma, chiasmatic/hypothalamic glioma, arachnoid cyst, pituitaryadenoma, and Rathke’s cleft cyst. Other lesions like hypothalamic hamartomas,histiocytosis-X, and epidermoids are less commonly seen.
Optic pathways tumors of childhood
Optic pathway gliomas represent 3% to 6% ofpediatric brain tumors and comprise a broad spectrum of tumors ranging fromtubular thickening of the optic nerves and chiasm to massive exophytic lesionsof the hypothalamus. Prechiasmatic Optic Glioma commonly presents asprogressive visual loss and, proptosis, if it involves the intraorbital nerve. DiffuseChiasmatic Glioma usually manifests as a combination of diminished visualacuity and bilateral visual field deficits. Endocrinopathy and hydrocephalusare rare. Most children have neurofibromatosis, and diffuse chiasmatic gliomamay be diagnosed.
Exophytic Chiasmatic-Hypothalamic Gliomaare a unique subset of posterior tumors that should be grouped separatelybecause they are amenable to radical surgical excision.These tumors may growdiffusely, expanding the third ventricle and displacing normal anatomylaterally or exophytically into the suprasellar and pre-mesencephalic cisterns.There are three distinct age-related patterns of clinical presentation inchildren with exophytic chiasmatic-hypothalamic gliomas.
Infants almost invariably present withmacrocephaly, failure to thrive, and visual failure. Failure to thrive may beassociated with the diencephalic syndrome.In children 2 to 5 years old,endocrine dysfunction is the most common presentation, including diabetesinsipidus, precocious or delayed puberty, and hypothyroidism.
Older children and young adults most oftenpresent with visual complaints and endocrinopathy. Surgery plays an importantrole in the management of the child with a chiasmatic or hypothalamic glioma.Surgery is often indicated to obtain histopathological confirmation of thediagnosis or when the tumor has a significant mass effect. Several groups haverecently reported on the effectiveness of radiation therapy in the managementof chiasmatic or hypothalamic gliomas.However, many patients with chiasmatic-hypothalamictumors are less than 3 years old, many groups have been using chemotherapy forthese tumors.
Suprasellar Germ Cell Tumor
Germ cell tumors are more common in Japanare rarely seen in India as well.
Craniopharyngioma
Craniopharyngiomas are the most commontumors of nonglial origin in pediatric populationDespite their benignhistological appearance, craniopharyngiomas commonly cause progressiveneurological deterioration and death because of their propensity to involvecritical structures as the hypothalamus, optic nerves, and pituitary gland. Thecyst contains cholesterol rich fluid. Intratumoral calcification is almostalways identified. Patients with craniopharyngiomas develop symptoms related totumor location.
The management of patients withcraniopharyngiomas remains controversial, with proponents for radicalresection, radiation therapy, or a combination of these modalities. Bestlong-term outcome has been observed with total surgical removal of the tumor.Therefore, the first approach to all craniopharyngiomas in children should beattempted total resection. If it is not possible because of its hazardoussequelae, a small tumor portion can be left in situ to avoid injury to vitalneurovascular structures. When subtotal excision is performed, the residualmass can be observed for any symptomatic recurrence which when appears can betreated with second attempt surgery or radiation therapy.
Pediatric Cerebral Hemispheric
The estimated incidence of cerebralhemispheric tumors in pediatric age group can be 6-8 cases per million childreneach year. Low-grade astrocytomas occur in all age groups, with the peakincidence between 8 and 12 years of age. High-grade astrocytomas are relativelyuncommon in children PNETs, althoughoccur in a wide age range, the mean age at diagnosis is 7 to 10 years. They aremost commonly located in the frontopariental region. Primary cerebralneuroblastoma is a distinct pathologic and clinical entity, and the mean age of
Gangliogliomas juvenile pilocytic astrocytoma andpleomorphic xanthoastrocytoma. Supratentorial ependymal Oligodendrogliomas Youngerchildren may present with increased head circumference, a bulging fontanelle,diplopia and strabismus caused by sixth nerve palsy, vomiting, or papilledema.Focal neurologic deficits include facial weakness, language deficits,hemiparesis, monoparesis, visual field defects, and asymmetric sensory loss. Seizuresare frequently associated with low-grade hemispheric glial tumors.11 CT and MRimaging are generally the methods of choice for the diagnosis of tumor. NewerMR techniques such as the MR spectroscopy, functional MRI, and MR tractography arevery useful in the diagnosis, treatment, and further evaluation of thesetumors. Generally, gross total resection of the tumor, when possible, is thebest treatment option for all hemispheric tumors in children Tumors that areinfiltrating in surrounding brain also pose great difficulties.
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