Clinico -Pathologic Conference Pediatrics

1. Clinico-Pathologic ConferencePediatrics Borela-Cotaoco 17 February 2010

2. Case summary

3. Course in the wards

4. Laboratories • Chest X-ray: normal • Chemistry

5. Laboratories • Blood Chemistry

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 heterogenoushyperdense lesion in the cerebellarvermis with perilesional edema and mass effect. Moderate extraventricular obstructive hydrocephalus.

8. differentials

9. Differentials • Pseudotumorcerebri • Medulloblastoma • Ependymoma • Brainstem glioma

10. PseudotumorCerebri • 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 cerebellarvermis on CT Scan

11. Medulloblastoma • Rule In • Headache • Vomiting • (+) Romberg’s sign • Nystagmus • Limited lateral eye movement on the left • Lesion in the cerebellarvermis on CT scan • Rule Out • Potential for metastasis

12. tuberculoma • RULE IN • Increased ICP • Infratentorial signs, espcerebellar • CT scan findings: lesion in the cerebellarvermis with perilesionaledema • 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 • 2nd most common childhood malignancy • Mortality as high as 45 % • 5 categories of tumors comprise 80 % of all brain tumors in children: 1. Juvenile pilocyticastrocytoma, 2. Medulloblastoma/primary neuroectodermaltumor 3. Diffuse astrocytomas 4. Ependymomas 5. Craniopharyngomas

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

18. Astrocytomas • the most common intracranial neoplasm • low grade astrocytomasoccuring mainly in childhood and with a excellent prognosis

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

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 pilocyticastrocytoma • 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 18th ed.

28. Thank you!