clinico-pathologic conference pediatrics borela-cotaoco 17 february 2010
TRANSCRIPT
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Clinico-Pathologic ConferencePediatrics
Borela-Cotaoco17 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
Calcium 2.62 (N: 8.8-10.5 mg/dl)Magnesium 1.0 (N:0.650-1.050 mmol/L)
Creatinine 61 (N: 59-104 umol/L)
Uric acid 281 (N: 0.160-0.43 mmol/L)
Sodium 143 (N: 135-144 mg/dl)
Potassium 3.7 (N: 3.6-5.2 mmol/L)
Chloride 105 (N:104-108 mmol/L)
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Laboratories• Blood Chemistry
4-4-09 4-9-09Hgb (N: 14-16 mg/dl) 141 128Hct (N: 0.40-0.54) 0.42 0.38Platelet count 260WBC 10.9Neu 0.66Lymphocytes 0.24Eosinophils 0.05
BasophilsStabs 0.01ESR (N: 0-15 mm/hr) 21Blood 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– 2nd 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 tumor3. Diffuse astrocytomas4. Ependymomas5. 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 18th ed.
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Thank you!