1. Clinico-Pathologic Conference Pediatrics Borela-Cotaoco 17 February 2010

2. Case summary

3. Course in the wards

4. 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)

5. 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+

6. 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

7. Laboratories Audiometry – unremarkable CT scan – Slight enhancing heterogenous hyperdense lesion in the cerebellar vermis with perilesional edema and mass effect. Moderate extraventricular obstructive hydrocephalus.

8. differentials

9. Differentials Pseudotumor cerebri Medulloblastoma Ependymoma Brainstem glioma

10. 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

11. 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

12. 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

13. Ependymoma RULE IN – Projectile vomiting – Hydrocephalus RULE OUT – Hyperdense lesion

14. Brainstem glioma RULE IN – Headache and vomiting – Age of the patient (10 y/o) – Horizontal nystagmus RULE OUT – No gait disturbances – No ataxia – Papilledema

15. Primary impression

16. 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

17. Brain tumors with a male predominance would be: Medulloblastoma and Ependymoma Epidemiology

18. Astrocytomas – the most common intracranial neoplasm – low grade astrocytomas occuring mainly in childhood and with a excellent prognosis

19. Associated environmental risk factors are not known, except for IONIZING RADIATION Loss of DNA on chromosomes 10p, 17p, 13q and 9. Etiology

20. 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

21. 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

22. – Others Headache Vomiting, with or without nausea No visual problems Anemia Hyperreflexia of the lower extremities No pathologic reflexes, supple neck

23. 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

24. imaging

25. Treatment Surgery Radiation therapy Chemotherapy

26. Prognosis After surgical resection – Complete: 80-100% overall survival rate – Partial: 50-95% overall survival rate Low metastatic potential Rarely invasive Leptomeningeal spread

27. References Nelson’s Textbook of Pediatrics 18 th ed.

28. Thank you!