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Clinico-Pathologic Conference Pediatrics Borela-Cotaoco 17 February 2010
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Case summary
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Course in the wards
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Laboratories Chest X-ray: normal Chemistry Calcium2.62 (N: 8.8-10.5 mg/dl) Magnesium1.0 (N:0.650-1.050 mmol/L) Creatinine61 (N: 59-104 umol/L) Uric acid281 (N: 0.160-0.43 mmol/L) Sodium143 (N: 135-144 mg/dl) Potassium3.7 (N: 3.6-5.2 mmol/L) Chloride105 (N:104-108 mmol/L)
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Laboratories Blood Chemistry 4-4-094-9-09 Hgb (N: 14-16 mg/dl)141128 Hct (N: 0.40-0.54)0.420.38 Platelet count260 WBC10.9 Neu0.66 Lymphocytes0.24 Eosinophils0.05 Basophils Stabs0.01 ESR (N: 0-15 mm/hr)21 Blood type B+
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Laboratories CSF (ventricular) – 5 cc of clear, colorless liquid – Ph: 7.5 – SG: 1.010 – RBC: 514 x 10^6 (N: 0-10 cells) – WBC: 1 x 10^6 – Total protein: 0.11 (N: 20-40 mg/dl) – Glucose: 4.7 (N: 60-160 mg/dl) – Pandy’s: negative MRI of the spine (Post-operative) – normal cervical, thoracic and lumbar spine
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Laboratories Audiometry – unremarkable CT scan – Slight enhancing heterogenous hyperdense lesion in the cerebellar vermis with perilesional edema and mass effect. Moderate extraventricular obstructive hydrocephalus.
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differentials
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Differentials Pseudotumor cerebri Medulloblastoma Ependymoma Brainstem glioma
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Pseudotumor Cerebri RULE IN – Overweight – Headache – Vomiting – Absent focal neurologic signs – Mentation and laertness preserved – Ataxia – Limited lateral eye movements – Normal CSF total protein content RULE OUT – Papilledema not mentioned – No visual field defect – Increased CSF RBC count – (+) hyperdense lesion in the cerebellar vermis on CT Scan
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Medulloblastoma Rule In – Headache – Vomiting – (+) Romberg’s sign – Nystagmus – Limited lateral eye movement on the left – Lesion in the cerebellar vermis on CT scan Rule Out – Potential for metastasis
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tuberculoma RULE IN – Increased ICP – Infratentorial signs, esp cerebellar – CT scan findings: lesion in the cerebellar vermis with perilesional edema RULE OUT – Clear CXR – No TB symptoms – (-) Kernig’s and Brudzinski’s signs
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Ependymoma RULE IN – Projectile vomiting – Hydrocephalus RULE OUT – Hyperdense lesion
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Brainstem glioma RULE IN – Headache and vomiting – Age of the patient (10 y/o) – Horizontal nystagmus RULE OUT – No gait disturbances – No ataxia – Papilledema
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Primary impression
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Epidemiology Brain tumors – 2 nd most common childhood malignancy – Mortality as high as 45 % – 5 categories of tumors comprise 80 % of all brain tumors in children: 1. Juvenile pilocytic astrocytoma, 2. Medulloblastoma/primary neuroectodermal tumor 3. Diffuse astrocytomas 4. Ependymomas 5. Craniopharyngomas
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Brain tumors with a male predominance would be: Medulloblastoma and Ependymoma Epidemiology
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Astrocytomas – the most common intracranial neoplasm – low grade astrocytomas occuring mainly in childhood and with a excellent prognosis
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Associated environmental risk factors are not known, except for IONIZING RADIATION Loss of DNA on chromosomes 10p, 17p, 13q and 9. Etiology
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Clinical Manifestations Progressive headache – No history of infection – Lack of fever, GI and urinary problems – Unremarkable laboratory work-up – Increasing intensity, frequency, association with vomiting, unresponsive to medications – ↑ ICP secondary to an underlying tumor
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Juvenile pilocytic astrocytoma – Localized signs and symptoms of cerebellar dysfunction Positive Romberg’s sign, intact motor strength, no gait disturbance Horizontal nystagmus, no auditory problems CT scan findings
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– Others Headache Vomiting, with or without nausea No visual problems Anemia Hyperreflexia of the lower extremities No pathologic reflexes, supple neck
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Diagnosis Biopsy – Bundles of compact fibrillary tissue with loose, microcystic spongy areas – Rosenthal fibers MRI – Contrast-enhancing nodule Lumbar Puncture -very strongly contraindicated Molecular/Cytogenetic and serum evaluation – Not necessary
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imaging
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Treatment Surgery Radiation therapy Chemotherapy
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Prognosis After surgical resection – Complete: 80-100% overall survival rate – Partial: 50-95% overall survival rate Low metastatic potential Rarely invasive Leptomeningeal spread
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References Nelson’s Textbook of Pediatrics 18 th ed.
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Thank you!
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