Clinico -Pathologic Conference Pediatrics
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Transcript of Clinico -Pathologic Conference Pediatrics
Clinico-Pathologic ConferencePediatrics
Borela-Cotaoco17 February 2010
Case summary
Course in the wards
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)
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+
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
Laboratories• Audiometry– unremarkable
• CT scan– Slight enhancing heterogenous hyperdense lesion in the
cerebellar vermis with perilesional edema and mass effect. Moderate extraventricular obstructive hydrocephalus.
differentials
Differentials• Pseudotumor cerebri• Medulloblastoma• Ependymoma• Brainstem glioma
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
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
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
Ependymoma• RULE IN– Projectile vomiting– Hydrocephalus
• RULE OUT– Hyperdense lesion
Brainstem glioma• RULE IN– Headache and vomiting– Age of the patient (10
y/o) – Horizontal nystagmus
• RULE OUT– No gait disturbances– No ataxia– Papilledema
Primary impression
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
• Brain tumors with a male predominance would be: Medulloblastoma and Ependymoma
Epidemiology
• Astrocytomas– the most common intracranial neoplasm– low grade astrocytomas occuring mainly in
childhood and with a excellent prognosis
• Associated environmental risk factors are not known, except for IONIZING RADIATION
• Loss of DNA on chromosomes 10p, 17p, 13q and 9.
Etiology
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
• 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
– Others• Headache• Vomiting, with or without nausea• No visual problems• Anemia• Hyperreflexia of the lower extremities• No pathologic reflexes, supple neck
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
imaging
Treatment• Surgery• Radiation therapy• Chemotherapy
Prognosis• After surgical resection– Complete: 80-100% overall survival rate– Partial: 50-95% overall survival rate
• Low metastatic potential• Rarely invasive• Leptomeningeal spread
References• Nelson’s Textbook of Pediatrics 18th ed.
Thank you!