Brain and Spinal Cord Tumors
 
 

Supratentorial Brain Tumors
Brain tumors are second most common neoplasm of childhood after leukaemia, primary brain tumors are responsible for 20% of all cancers in children, and for 20% of childhood cancer deaths. The yearly incidence of childhood brain tumors is reported to be 24 per million children less than 15 years of age. The neurologic functional outcome and quality of life of children is goal of treatment .

Classification of primary brain tumors in children
Brain tumors are classified primarily by their location [supratentorial or infratentorial] and histology. The supratentorial tumors can be sellar, suprasellar, or hemispheric tumors [intraventricular or parenchymal]. The most recent World Health organization (WHO) classification schema for tumors of the CNS is done with  criteria of histologic typing of tumors.

Clinical features of childhood brain tumors
Presenting signs and symptoms of primary brain tumors vary by age and location. Tumors that present in infancy may be more insidious because of the non-specific nature of clinical features that arise from a variety of causes, including vomiting, irritability, listlessness, macrocephaly, failure to thrive, and stagnation of development or loss of developmental milestones. Common signs and symptoms of brain tumors in children include Irritability, Listlessness Vomiting, Headache, Failure to thrive, Macrocephaly, Seizures, Focal neurologic deficits/ hemiparesis, Visual field loss/ deficit, Neuroendocrine dysfunction.

Suprasellar Tumors
The sellar and suprasellar region comprise a heterogenous group of lesions that can cause a number of neurological and endocrinological disturbances by virtue of their proximity to the optic apparatus, 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, pituitary adenoma, 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% of pediatric brain tumors and comprise a broad spectrum of tumors ranging from tubular thickening of the optic nerves and chiasm to massive exophytic lesions of the hypothalamus. Prechiasmatic Optic Glioma commonly presents as progressive visual loss and, proptosis, if it involves the intraorbital nerve. Diffuse Chiasmatic Glioma usually manifests as a combination of diminished visual acuity and bilateral visual field deficits. Endocrinopathy and hydrocephalus are rare. Most children have neurofibromatosis, and diffuse chiasmatic glioma may be diagnosed.
Exophytic Chiasmatic-Hypothalamic Glioma are a unique subset of posterior tumors that should be grouped separately because they are amenable to radical surgical excision.These tumors may grow diffusely, expanding the third ventricle and displacing normal anatomy laterally or exophytically into the suprasellar and pre-mesencephalic cisterns. There are three distinct age-related patterns of clinical presentation in children with exophytic chiasmatic-hypothalamic gliomas.

Infants almost invariably present with macrocephaly, failure to thrive, and visual failure. Failure to thrive may be associated with the diencephalic syndrome.In children 2 to 5 years old, endocrine dysfunction is the most common presentation, including diabetes insipidus, precocious or delayed puberty, and hypothyroidism.

Older children and young adults most often present with visual complaints and endocrinopathy. Surgery plays an important role in the management of the child with a chiasmatic or hypothalamic glioma. Surgery is often indicated to obtain histopathological confirmation of the diagnosis or when the tumor has a significant mass effect. Several groups have recently reported on the effectiveness of radiation therapy in the management of chiasmatic or hypothalamic gliomas.However, many patients with chiasmatic-hypothalamic tumors are less than 3 years old, many groups have been using chemotherapy for these tumors.

Suprasellar Germ Cell Tumor
Germ cell tumors are more common in Japan are rarely seen in India as well.

Craniopharyngioma
Craniopharyngiomas are the most common tumors of nonglial origin in pediatric populationDespite their benign histological appearance, craniopharyngiomas commonly cause progressive neurological deterioration and death because of their propensity to involve critical structures as the hypothalamus, optic nerves, and pituitary gland. The cyst contains cholesterol rich fluid. Intratumoral calcification is almost always identified. Patients with craniopharyngiomas develop symptoms related to tumor location.
The management of patients with craniopharyngiomas remains controversial, with proponents for radical resection, radiation therapy, or a combination of these modalities. Best long-term outcome has been observed with total surgical removal of the tumor. Therefore, the first approach to all craniopharyngiomas in children should be attempted total resection. If it is not possible because of its hazardous sequelae, a small tumor portion can be left in situ to avoid injury to vital neurovascular structures. When subtotal excision is performed, the residual mass can be observed for any symptomatic recurrence which when appears can be treated with second attempt surgery or radiation therapy.

Pediatric Cerebral Hemispheric
The estimated incidence of cerebral hemispheric tumors in pediatric age group can be 6-8 cases per million children each year. Low-grade astrocytomas occur in all age groups, with the peak incidence between 8 and 12 years of age. High-grade astrocytomas are relatively uncommon in children  PNETs, although occur in a wide age range, the mean age at diagnosis is 7 to 10 years. They are most commonly located in the frontopariental region. Primary cerebral neuroblastoma is a distinct pathologic and clinical entity, and the mean age of

Gangliogliomas juvenile pilocytic astrocytoma and pleomorphic xanthoastrocytoma. Supratentorial ependymal Oligodendrogliomas Younger children 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. Seizures are frequently associated with low-grade hemispheric glial tumors.11 CT and MR imaging are generally the methods of choice for the diagnosis of tumor. Newer MR techniques such as the MR spectroscopy, functional MRI, and MR tractography are very useful in the diagnosis, treatment, and further evaluation of these tumors. Generally, gross total resection of the tumor, when possible, is the best treatment option for all hemispheric tumors in children Tumors that are infiltrating in surrounding brain also pose great difficulties.

 
Clinic at Mulund (West)
 

         
    M.B.B.S.
M.S.(General Surgery)
D.N.B.(General Surgery)
M.CH.(Neuro Surgery)
D.N.B.(Neuro Surgery)
M.N.A.M.S
 
         
         
 
  Fellowship in Pediatric
Neurosurgery from Tel
AVIV & Great Ormand
Street Hospital for Sick
Children, London, U.K.
 
         
         
 
  Registration No.:
83299
(Maharashtra Medical
Council)