CASE PRESENTATION By Dr. Ayman S.Iskander History Name : Aziza Ahmed Mohmed Makaran . Age : 4 years- 10 months . Sex : Female . Nationality : Somali Complaint: Repeated vomiting , colic 3 days duration ?? Hx of fever (not documented) Condition started 3 days prior to her admission by vomiting , colic mainly in epigastric region. Unclear history of fever, headache. Child was product of C.S. with normal neonatal history. No history of contact to sick person . No history of previous admission. No history of head trauma. No history suggestive of food poisoning . No history of drug intake. EXAMINATION Vital signs : Temp. : 37 c HR : 120/m RR : 30/m O2 SAT : 97% Bl P 110/70 Patient did not look well, but conscious Chest , Heart clinically free ENT :free Abdomen : soft ,lax with some epigastric tenderness . Genitalia : normal female genitalia CNS : slighitly drowsy ,coscious . no signs of meningial irritation . no hyper or hypotonia, normal DTR reflexes . pupil : round, regular and reactive Apparent convergent squint . Gait : she is bed ridden, very irritable and refusing to move Fundus : Bilateral papiledema and retinal hemorrhage . Progress in Hospital Brought to Emergency Dept on 17-09-1012 at afternoon with vomiting, colic 3 days , giving IVF for 2 hours but still tired, vomiting colic not improved . Admitted to the hospital at 09:00 pm To continue IVF , I V. Zantac At 7 am of next day after 8 hrs from admission she had severe attack of vomiting ,??convulsion in sort of staring look ,without tonic or clonic components. Vital sign was o2 sat 98% , HR :60/min , Bp : 131/78 , and drowsy . CT brain urgently requested and done . An Uregent CT brain FINDINGS " Well defined hyper-dense spherical-shaped lesion in the cerebellar vermis, floor of the 4th ventricle, showing enhancement in the post-contrast images. This lesion has central calcification and areas of necrosis, and demonstrates mass effect in terms of compressing the 4th ventricle with mild dilatation of the distal ventricular system. " The cerebral cortical hemispheres demonstrate normal gyri with preserved grey/white matter differentiation. " Normal attenuation and configuration of the basal ganglia, internal and external capsules. " Normal attenuation of the peri-ventricular white matter and centrum semi-oval. " No midline shift. " Normal calvarium bones. CONCLUSION " Well defined hyper-attenuated lesion in the cerebellar vermis with central areas of calcification and necrosis associated with mild hydrocephalus. The patient age and CT features are suggestive of Medulloblastoma. Then neurosurgeon contacted and he adviced to send the case to Neurosurgery Dept, Ibin Sina Hospital and patient admitted their same morning. BRAIN TUMORS IN PEDIATRICS Cancer in Children <15 years old: Major Histologic Subtypes 11% 5% 32% 6% 7% 8% 11% 20% Leukemia CNS tumors Lymphomas Neuroblastoma Sarcomas Wilms tumor Bone tumors Misc Pediatric Brain Tumors Second most common malignancy of childhood (~20% of all childhood cancers) Leading Cause of Cancer Death in Children Incidence in developed countries is 32.7-34.9 cases per million Classification of brain tumors in pediatrics Intracranial neoplasms Primary Secondary Metastatic Local invasion Tumors of the spinal cord Primary brain tumors Primary brain tumors are rare 2.5% of all cancer deaths Second most common type of tumor in children There are over 100 different brain tumors Most common types Astrocytomas Medulloblastomas Grades I-IV primitive neuroectodermal tumor-PNET Meningiomas Pituitary adenomas Clinical presentation Clinical symptoms depend upon: Age, location, and type of tumor and grade Symptoms may include: Increased intracranial pressure secondary to obstruction of CSF at aqueduct hydrocephalus (infants), headache, papilledema, vomiting seizures focal neurological deficits hormonal changes (pituitary adenoma) visual changes (diplopia, field defects) Pituitary adenoma - pressure on optic chiasm Medulloblastoma the most common malignant brain tumor in children, accounting for 10-20% of primary CNS neoplasms and approximately 40% of all posterior fossa tumors. It is a highly invasive embryonal neuroepithelial tumor that arises in the cerebellum and has a tendency to disseminate throughout the CNS early in its course Epidemiology Posterior fossa tumors are more common in children than the adults. Between 54% and 70% of all childhood brain tumors originate in the posterior fossa. Peak age of incidence is 5-7 years Approximately 300 new cases in the US/year ~ 5/1 million children 2:1 Male predominence 75% diagnosed <15 years of age No associated environmental factors known Clinical presentation Although 70-90% of patients with medulloblastomas present with a history of headaches, emesis, and lethargy, these symptoms are generally intermittent and subtle. Duration of symptoms for 3 months or more before diagnosis is common. Increased intracranial pressure (ICP) Early symptoms are secondary to increased ICP. The classic triad consists of morning headaches, vomiting, and lethargy. Cushing triad (ie, hypertension , bradycardia, and hypoventilation), an uncommon finding in children with increased intracranial pressure, usually indicates impending herniation. Initial signs of increased ICP are usually subacute, nonspecific, and nonlocalizing. School-aged children may complain of vague intermittent headaches and fatigue. Infants may present with irritability, anorexia, and developmental delay. Cerebellar dysfunction With increasing tumor size and invasion into the surrounding brain tissue, more characteristic symptoms appear. One symptom is progressively worsening ataxia involving the lower extremities Brain stem deficits Tumor infiltration of the brain stem or increased ICP may result in diplopia and multiple other cranial nerve findings, such as facial weakness, tinnitus, hearing loss, head tilt, and stiff neck. Metastatic disease Uncommon Causes Environmental Epidemiological studies investigating parental occupational exposures, environmental exposures and maternal nutritional intake have not proven a direct link Familial and heritable disease Medulloblastoma is associated with recessively inherited Turcot and ataxia-telangiectasia syndromes. Genetic associations The most frequent cytogenetic abnormality in sporadic medulloblastoma is an isochromosome 17q [i(17q)]. Of tumors analyzed, 40-50% have a deletion of the short arm of chromosome 17, implicating the presence of a tumor suppressor gene that maps to 17p, which is distinct from the p53 gene. Alternatively, a gene on 17q may be related to transformation because of increased copy number. Current Risk Stratification Average Risk Age > 3 years M0 at diagnosis Less than 1.5cm3 of residual tumor on post-operative MRI High Risk Age < 3 years M1-M4 stage at diagnosis Treatment Surgery: Goal is for gross total resection of the primary mass Radiation: Craniospinal with a posterior fossa boost: Currently trying to safely reduce dose and field Chemotherapy: Platinum based regimens. (Allows for lower dose radiation in M0 patients Prognosis according to Risk Group < 3 years of age at diagnosis: <30 % chance of cure >3 years with localized disease: 80% chance of cure (goal= decrease toxicity) >3 years with metastatic disease: ~ 50% chance of cure (increase cure) Sisters having fun together: Life after brain tumor treatment References Giangaspero F, Eberhart CG, Haapasalo H, Pietsch T, Wiestler OD, et al. (2007) Medulloblastoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, eds. WHO classification of tumours of the central nervous system. Lyon: IARC .Press. pp 132–140. 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